Nakamura Hideki
| Faculty of Medicine Research Center for Cooperative Projects | Specially Appointed Research Associate |
Last Updated :2025/06/07
■Researcher basic information
Researchmap personal page
J-Global ID
Educational Organization
- Bachelor's degree program, Departments of Medicine, School of Medicine
- Master's degree program, Graduate School of Medicine
- Doctoral (PhD) degree program, Graduate School of Medicine
■Research activity information
Papers
- Wnt/β-cateninシグナルが表皮角化細胞のヘミデスモソームを制御する
小住 英之, 野原 拓馬, 藤村 悠, 岩田 浩明, 中村 秀樹, 氏家 英之, 夏賀 健, 渡邉 美佳, Donati Giacomo, 新熊 悟, 築山 忠維
日本皮膚科学会雑誌, 132, 12, 2697, 2697, (公社)日本皮膚科学会, Nov. 2022
Japanese - Wnt/β-Catenin Signaling Stabilizes Hemidesmosomes in Keratinocytes.
Hideyuki Kosumi, Mika Watanabe, Satoru Shinkuma, Takuma Nohara, Yu Fujimura, Tadasuke Tsukiyama, Giacomo Donati, Hiroaki Iwata, Hideki Nakamura, Hideyuki Ujiie, Ken Natsuga
The Journal of investigative dermatology, 142, 6, 1576, 1586, Jun. 2022, [International Magazine]
English, Scientific journal, Hemidesmosomes (HDs) are adhesion complexes that promote epithelial-stromal attachment in stratified and complex epithelia, including the epidermis. In various biological processes, such as differentiation and migration of epidermal keratinocytes during wound healing or carcinoma invasion, quick assembly and disassembly of HDs are prerequisites. In this study, we show that inhibition of Wnt/β-catenin signaling disturbs HD organization in keratinocytes. Screening with inhibitors identified the depletion of HD components and HD-like structures through Wnt inhibition, but keratinocyte differentiation was not affected. Wnt inhibition significantly diminished plectin and type XVII collagen expression in the basal side of Wnt-inhibited cells and the dermo-epidermal junction of the Wnt-inactive murine basal epidermis. Similar to Wnt inhibition, PLEC-knockout cells or cells with plectin-type XVII collagen binding defects showed type XVII collagen reduction in the basal side of the cells, implying the possible involvement of Wnt/β-catenin signaling in HD assembly. Atypical protein kinase C inhibition ameliorated the phenotypes of Wnt-inhibited cells. These findings show that Wnt/β-catenin signaling regulates the localization of HD components in keratinocytes and that the atypical protein kinase C pathway is involved in Wnt inhibition‒induced HD disarrangement. Our study suggests that the Wnt signaling pathway could be a potential therapeutic target for treating HD-defective diseases, such as epidermolysis bullosa. - Cas9-guided haplotyping of three truncation variants in autosomal recessive disease.
Ken Natsuga, Yoshikazu Furuta, Shota Takashima, Takuma Nohara, Hsin-Yu Huang, Satoru Shinkuma, Hideki Nakamura, Yousuke Katsuda, Hideaki Higashi, Chao-Kai Hsu, Satoshi Fukushima, Hideyuki Ujiie
Human mutation, 43, 7, 877, 881, 21 Apr. 2022, [International Magazine]
English, Scientific journal, An autosomal recessive disease is caused by biallelic loss-of-function mutations. However, when more than two disease-causing variants are found in a patient's gene, it is challenging to determine which two of the variants are responsible for the disease phenotype. Here, to decipher the pathogenic variants by precise haplotyping, we applied nanopore Cas9-targeted sequencing (nCATS) to three truncation COL7A1 variants detected in a patient with recessive dystrophic epidermolysis bullosa (EB). The distance between the most 5' and 3' variants was approximately 19 kb at the level of genomic DNA. nCATS successfully demonstrated that the most 5' and 3' variants were located in one allele while the variant in between was located in the other allele. Interestingly, the proband's mother, who was phenotypically intact, was heterozygous for the allele that harbored the two truncation variants, which could otherwise be misinterpreted as those of typical recessive dystrophic EB. Our study highlights the usefulness of nCATS as a tool to determine haplotypes of complicated genetic cases. Haplotyping of multiple variants in a gene can determine which variant should be therapeutically targeted when nucleotide-specific gene therapy is applied. - 成人栄養障害型表皮水疱症患者を対象としたMuse細胞製品CL2020の国内1/2相試験 52週までの安全性の検討
藤田 靖幸, 野原 拓馬, 高島 翔太, 夏賀 健, 中村 秀樹, 清水 宏, 足立 太起, 吉田 憲司, 石河 晃, 新熊 悟, 武市 拓也, 秋山 真志, 和田 理
日本皮膚科学会雑誌, 132, 3, 510, 510, (公社)日本皮膚科学会, Mar. 2022
Japanese - 表皮下水疱は毛包の成長を犠牲にして治癒する
藤村 悠, 高島 翔太, 中村 秀樹, 小住 英之, 王 禹楠, 眞井 洋輔, 氏家 英之, 岩田 浩明, 西江 渉, 清水 宏, 夏賀 健, 渡邉 美佳, 大野 航太, 小林 康明, 長山 雅晴, Andrea Lauria, Valentina Proserpio, Salvatore Oliviero, Giacomo Donati
日本皮膚科学会雑誌, 131, 9, 2071, 2071, (公社)日本皮膚科学会, Aug. 2021
Japanese - Hair follicle stem cell progeny heal blisters while pausing skin development
Yu Fujimura, Mika Watanabe, Kota Ohno, Yasuaki Kobayashi, Shota Takashima, Hideki Nakamura, Hideyuki Kosumi, Yunan Wang, Yosuke Mai, Andrea Lauria, Valentina Proserpio, Hideyuki Ujiie, Hiroaki Iwata, Wataru Nishie, Masaharu Nagayama, Salvatore Oliviero, Giacomo Donati, Hiroshi Shimizu, Ken Natsuga
EMBO reports, 22, 7, EMBO, 05 Jul. 2021
Scientific journal - Zonula occludens-1 demonstrates a unique appearance in buccal mucosa over several layers.
Keisuke Imafuku, Mayumi Kamaguchi, Ken Natsuga, Hideki Nakamura, Hiroshi Shimizu, Hiroaki Iwata
Cell and tissue research, 384, 3, 691, 702, Jun. 2021, [International Magazine]
English, Scientific journal, Tight junctions (TJs) firmly seal epithelial cells and are key players in the epithelial barrier. TJs consist of several proteins, including those of the transmembrane claudin family and the scaffold zonula occludens (ZO) family. Epithelial tissues are exposed to different conditions: to air in the stratified epithelium of the skin and to liquids in the monolayer of the intestine. The TJs in stratified oral mucosal epithelium have remained insufficiently elucidated in terms of distributions, appearances and barrier functions of TJ proteins in normal buccal mucosa. We investigated these and ZO-1 and claudin-1 were found to be expressed in the top third and in the bottom three quarters of the mucosal epithelium. ZO-1 in the buccal mucosa was found to have an irregular linear appearance. ZO-1 in the buccal mucosa continuously existed in several layers. Electron microscopy revealed the buccal mucosa to have kissing points. In a biotin permeation assay that sought to investigate inside-outside barrier function, the biotin tracer penetrated several ZO-1 layers but did not pass through all the ZO-1 layers. We found that the oral mucosal cell knockdown of TJP1 or CLDN1 resulted in decreases of TER but no significant change in FITC-dextran leakage. Our results suggest that the distribution and appearance of ZO-1 in the buccal mucosa differ from those in the skin. We were unable to prove barrier function in this study but we did show barrier function against small molecules in vivo and against ions in vitro. - Intravenous allogeneic multilineage-differentiating stress-enduring cells in adults with dystrophic epidermolysis bullosa: a phase 1/2 open-label study.
Y Fujita, T Nohara, S Takashima, K Natsuga, M Adachi, K Yoshida, S Shinkuma, T Takeichi, H Nakamura, O Wada, M Akiyama, A Ishiko, H Shimizu
Journal of the European Academy of Dermatology and Venereology : JEADV, 03 Mar. 2021, [International Magazine]
English - Cultured Epidermal Autografts from Clinically Revertant Skin as a Potential Wound Treatment for Recessive Dystrophic Epidermolysis Bullosa.
Matsumura W, Fujita Y, Shinkuma S, Suzuki S, Yokoshiki S, Goto H, Hayashi H, Ono K, Inoie M, Takashima S, Nakayama C, Nomura T, Nakamura H, Abe R, Sato N, Shimizu H
The Journal of investigative dermatology, 139, 10, 2115, 2124.e11, Oct. 2019, [Peer-reviewed] - Electron Microscopic and Immunohistochemical Findings of the Epidermal Basement Membrane in Two Families with Nail-patella Syndrome.
Shinkuma S, Nakamura H, Maehara M, Takashima S, Nomura T, Fujita Y, Hasegawa S, Sato-Matsumura KC, Abe R, Shimizu H
Acta dermato-venereologica, 99, 12, 1110, 1115, Sep. 2019, [Peer-reviewed] - CRISPR/Cas9システムを用いた劣性栄養障害型表皮水疱症(RDEB)の治療法の開発
高島 翔太, 乃村 俊史, 氏家 英之, 夏賀 健, 岩田 浩明, 中村 秀樹, 清水 宏, 新熊 悟, 阿部 理一郎, 藤田 靖幸
日本皮膚科学会雑誌, 129, 9, 1909, 1909, (公社)日本皮膚科学会, Aug. 2019
Japanese - Case of autoimmune intraepidermal and subepidermal blistering disease in which autoantibodies to desmoglein 1 and BP230 coexist.
Sugai T, Ujiie H, Nakamura H, Kikuchi K, Iwata H, Shimizu H
The Journal of dermatology, 46, 6, e215, e216, Jun. 2019, [Peer-reviewed], [International Magazine]
English, Scientific journal - Efficient gene reframing therapy for recessive dystrophic epidermolysis bullosa using CRISPR/Cas9.
Takashima S, Shinkuma S, Fujita Y, Nomura T, Ujiie H, Natsuga K, Iwata H, Nakamura H, Vorobyev A, Abe R, Shimizu H
The Journal of investigative dermatology, 139, 8, 1711, 1721.e4, Mar. 2019, [Peer-reviewed]
Scientific journal - Congenital nevi with hypomelanosis and fine scales.
Natsuga K, Oiso N, Kurokawa I, Tsubura A, Nakamura H, Maya Y, Nishie W, Kawada A, Shimizu H
European journal of dermatology : EJD, 29, 1, 45, 48, Feb. 2019, [Peer-reviewed] - Novel COL7A1 mutation in a family with bullous dermolysis of the newborn: Phenotypic variability associated with a COL7A1 mutation within the same family.
Shota Takashima, Satoru Shinkuma, Yasuyuki Fujita, Ken Natsuga, Toshifumi Nomura, Tokimasa Hida, Shuku Ishikawa, Hideki Nakamura, Riichiro Abe, Hiroshi Shimizu
The Journal of dermatology, 45, 9, e260-e261, e261, Sep. 2018, [Peer-reviewed], [International Magazine]
English, Scientific journal - Ramucirumab-induced Multiple Haemangiomas of the Skin: Two Case Reports.
Kosumi H, Nishie W, Sugai T, Toyonaga E, Yoshimoto N, Nakamura H, Horibe R, Kitamura Y, Nakatsumi H, Shimizu H
Acta dermato-venereologica, 98, 4, 454, 455, Dec. 2017, [Peer-reviewed], [International Magazine]
English, Scientific journal - Superior Microvascular Perfusion of Infused Liposome-Encapsulated Hemoglobin Prior to Reductions in Infarctions after Transient Focal Cerebral Ischemia
Daisuke Shimbo, Takeo Abumiya, Kota Kurisu, Toshiya Osanai, Hideo Shichinohe, Naoki Nakayama, Ken Kazumata, Hideki Nakamura, Hiroshi Shimuzu, Kiyohiro Houkin
JOURNAL OF STROKE & CEREBROVASCULAR DISEASES, 26, 12, 2994, 3003, Dec. 2017, [Peer-reviewed]
English, Scientific journal - Loss of interaction between plectin and type XVII collagen results in epidermolysis bullosa simplex
Ken Natsuga, Wataru Nishie, Machiko Nishimura, Satoru Shinkuma, Mika Watanabe, Kentaro Izumi, Hideki Nakamura, Yoshiaki Hirako, Hiroshi Shimizu
HUMAN MUTATION, 38, 12, 1666, 1670, Dec. 2017, [Peer-reviewed]
English, Scientific journal - A case of recessive dystrophic epidermolysis bullosa with a novel c.6885_6898de114 mutation in the COL7A1 gene
Satoru Shinkuma, Tae Masunaga, Saori Miyawaki, Shota Takashima, Ken Natsuga, Toshifumi Nomura, Yasuyuki Fujita, Hideki Nakamura, Hiroshi Shimizu
JOURNAL OF DERMATOLOGICAL SCIENCE, 88, 1, 139, 141, Oct. 2017, [Peer-reviewed]
English - Type XVII collagen coordinates proliferation in the interfollicular epidermis
Mika Watanabe, Ken Natsuga, Wataru Nishie, Yasuaki Kobayashi, Giacomo Donati, Shotaro Suzuki, Yu Fujimura, Tadasuke Tsukiyama, Hideyuki Ujiie, Satoru Shinkuma, Hideki Nakamura, Masamoto Murakami, Michitaka Ozaki, Masaharu Nagayama, Fiona M. Watt, Hiroshi Shimizu
ELIFE, 6, Jul. 2017, [Peer-reviewed]
English, Scientific journal - The first familial cases of epidermolysis bullosa simplex, generalized severe with p.Asn176Ser in KRT5 revealing the clinical chronology
T. Sugai, S. Shinkuma, K. Inafuku, S. Takashima, T. Nomura, Y. Fujita, H. Nakamura, H. Shimizu
JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY, 31, 5, e251, e253, May 2017
English - Revertant Mosaicism in Ichthyosis with Confetti Caused by a Frameshift Mutation in KRT1
Shotaro Suzuki, Toshifumi Nomura, Toshinari Miyauchi, Masae Takeda, Hideki Nakamura, Satoru Shinkuma, Yasuyuki Fujita, Masashi Akiyama, Hiroshi Shimizu
JOURNAL OF INVESTIGATIVE DERMATOLOGY, 136, 10, 2093, 2095, Oct. 2016, [Peer-reviewed]
English - Transarterial Regional Brain Hypothermia Inhibits Acute Aquaporin-4 Surge and Sequential Microvascular Events in Ischemia/Reperfusion Injury
Kota Kurisu, Takeo Abumiya, Hideki Nakamura, Daisuke Shimbo, Hideo Shichinohe, Naoki Nakayama, Ken Kazumata, Hiroshi Shimizu, Kiyohiro Houkin
NEUROSURGERY, 79, 1, 125, 134, Jul. 2016, [Peer-reviewed]
English, Scientific journal - Epitope-Dependent Pathogenicity of Antibodies Targeting a Major Bullous Pemphigoid Autoantigen Collagen XVII/BP180
Mayumi Wada, Wataru Nishie, Hideyuki Ujiie, Kentaro Izumi, Hiroaki Iwata, Ken Natsuga, Hideki Nakamura, Yoshimasa Kitagawa, Hiroshi Shimizu
JOURNAL OF INVESTIGATIVE DERMATOLOGY, 136, 5, 938, 946, May 2016, [Peer-reviewed]
English, Scientific journal - Extracellular cleavage of collagen XVII is essential for correct cutaneous basement membrane formation
Machiko Nishimura, Wataru Nishie, Yoshinori Shirafuji, Satoru Shinkuma, Ken Natsuga, Hideki Nakamura, Daisuke Sawamura, Keiji Iwatsuki, Hiroshi Shimizu
HUMAN MOLECULAR GENETICS, 25, 2, 328, 339, Jan. 2016, [Peer-reviewed]
English, Scientific journal - Context-Dependent Regulation of Collagen XVII Ectodomain Shedding in Skin
Wataru Nishie, Ken Natsuga, Hiroaki Iwata, Kentaro Izumi, Hideyuki Ujiie, Ellen Toyonaga, Hiroo Hata, Hideki Nakamura, Hiroshi Shimizu
AMERICAN JOURNAL OF PATHOLOGY, 185, 5, 1361, 1371, May 2015, [Peer-reviewed]
English, Scientific journal - Skipped exon in COL7A1 determines the clinical phenotypes of dystrophic epidermolysis bullosa.
Toyonaga E, Nishie W, Komine M, Murata S, Shinkuma S, Natsuga K, Nakamura H, Ohtsuki M, Shimizu H
The British journal of dermatology, 172, 4, 1141, 1144, Apr. 2015, [Peer-reviewed]
Toyonaga E, Nishie W, Komine M, Murata S, Shinkuma S, Natsuga K, Nakamura H, Ohtsuki M, Shimizu H, The British journal of dermatology, 2014 - Post-ischemic Trans-arterial Regional Hypothermia Therapy Attenuates Microvascular Narrowing due to Astrocyte End-feet Swelling and Inhibits Subsequent Inflammatory Reactions in Ischemia Reperfusion Injury
Kurisu Kota, Abumiya Takeo, Nakamura Hideki, Shimbo Daisuke, Kazumata Ken, Nakayama Naoki, Shichinohe Hideo, Hokari Masaaki, Osanai Toshiya, Shimizu Hiroshi, Houkin Kiyohiro
STROKE, 46, Feb. 2015, [Peer-reviewed] - Bullous Pemphigoid Autoantibodies Directly Induce Blister Formation without Complement Activation
Hideyuki Ujiie, Tetsumasa Sasaoka, Kentaro Izumi, Wataru Nishie, Satoru Shinkuma, Ken Natsuga, Hideki Nakamura, Akihiko Shibaki, Hiroshi Shimizu
JOURNAL OF IMMUNOLOGY, 193, 9, 4415, 4428, Nov. 2014, [Peer-reviewed]
English, Scientific journal - 新規水疱性類天疱瘡マウスモデルの樹立と解析
氏家 英之, 芝木 晃彦, 西江 渉, 澤村 大輔, 王 剛, 立石 八寿貴, 李 強, 守内 玲寧, 喬 洪江, 中村 秀樹, 秋山 真志, 清水 宏
日本皮膚科学会雑誌, 122, 2, 379, 379, (公社)日本皮膚科学会, Feb. 2012
Japanese - A founder effect of c.1938delC in ITGB4 underlies junctional epidermolysis bullosa and its application for prenatal testing.
Natsuga K, Nishie W, Shinkuma S, Nakamura H, Arita K, Yoneda K, Kusaka T, Yanagihara T, Kosaki R, Sago H, Akiyama M, Shimizu H
Experimental dermatology, 20, 1, 74, 76, Jan. 2011, [Peer-reviewed] - Complete Paternal Isodisomy of Chromosome 17 in Junctional Epidermolysis Bullosa with Pyloric Atresia
Ken Natsuga, Wataru Nishie, Ken Arita, Satoru Shinkuma, Hideki Nakamura, Shogo Kubota, Sumihisa Imakado, Masashi Akiyama, Hiroshi Shimizu
JOURNAL OF INVESTIGATIVE DERMATOLOGY, 130, 11, 2671, 2674, Nov. 2010, [Peer-reviewed]
English - Plectin deficiency leads to both muscular dystrophy and pyloric atresia in epidermolysis bullosa simplex.
Natsuga K, Nishie W, Shinkuma S, Arita K, Nakamura H, Ohyama M, Osaka H, Kambara T, Hirako Y, Shimizu H
Human mutation, 31, E1687, 98, 10, Oct. 2010, [Peer-reviewed] - Bone marrow transplantation restores epidermal basement membrane protein expression and rescues epidermolysis bullosa model mice
Yasuyuki Fujita, Riichiro Abe, Daisuke Inokuma, Mikako Sasaki, Daichi Hoshina, Ken Natsuga, Wataru Nishie, James R. McMillan, Hideki Nakamura, Tadamichi Shimizu, Masashi Akiyama, Daisuke Sawamura, Hiroshi Shimizu
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 107, 32, 14345, 14350, Aug. 2010, [Peer-reviewed]
English, Scientific journal - Prevalent LIPH Founder Mutations Lead to Loss of P2Y5 Activation Ability of PA-PLA(1)alpha in Autosomal Recessive Hypotrichosis
Satoru Shinkuma, Masashi Akiyama, Asuka Inoue, Junken Aoki, Ken Natsuga, Toshifumi Nomura, Ken Arita, Riichiro Abe, Kei Ito, Hideki Nakamura, Hideyuki Ujiie, Akihiko Shibaki, Hiraku Suga, Yuichiro Tsunemi, Wataru Nishie, Hiroshi Shimizu
HUMAN MUTATION, 31, 5, 602, 610, May 2010, [Peer-reviewed]
English, Scientific journal - Plectin Expression Patterns Determine Two Distinct Subtypes of Epidermolysis Bullosa Simplex
Ken Natsuga, Wataru Nishie, Masashi Akiyama, Hideki Nakamura, Satoru Shinkuma, James R. McMillan, Akari Nagasaki, Cristina Has, Takeshi Ouchi, Akira Ishiko, Yoshiaki Hirako, Katsushi Owaribe, Daisuke Sawamura, Leena Bruckner-Tuderman, Hiroshi Shimizu
HUMAN MUTATION, 31, 3, 308, 316, Mar. 2010, [Peer-reviewed]
English, Scientific journal - Blockade of Autoantibody-Initiated Tissue Damage by Using Recombinant Fab Antibody Fragments against Pathogenic Autoantigen
Gang Wang, Hideyuki Ujiie, Akihiko Shibaki, Wataru Nishie, Yasuki Tateishi, Kazuhiro Kikuchi, Qiang Li, James R. McMillan, Hiroshi Morioka, Daisuke Sawamura, Hideki Nakamura, Hiroshi Shimizu
AMERICAN JOURNAL OF PATHOLOGY, 176, 2, 914, 925, Feb. 2010, [Peer-reviewed]
English, Scientific journal - A Novel Active Mouse Model for Bullous Pemphigoid Targeting Humanized Pathogenic Antigen
Hideyuki Ujiie, Akihiko Shibaki, Wataru Nishie, Daisuke Sawamura, Gang Wang, Yasuki Tateishi, Qiang Li, Reine Moriuchi, Hongjiang Qiao, Hideki Nakamura, Masashi Akiyama, Hiroshi Shimizu
JOURNAL OF IMMUNOLOGY, 184, 4, 2166, 2174, Feb. 2010, [Peer-reviewed]
English, Scientific journal - Keratinocyte-/Fibroblast-Targeted Rescue of Col7a1-Disrupted Mice and Generation of an Exact Dystrophic Epidermolysis Bullosa Model Using a Human COL7A1 Mutation
Kei Ito, Daisuke Sawamura, Maki Goto, Hideki Nakamura, Wataru Nishie, Kaori Sakai, Ken Natsuga, Satoru Shinkuma, Akihiko Shibaki, Jouni Uitto, Christopher P. Denton, Osamu Nakajima, Masashi Akiyama, Hiroshi Shimizu
AMERICAN JOURNAL OF PATHOLOGY, 175, 6, 2508, 2517, Dec. 2009, [Peer-reviewed]
English, Scientific journal - Possible involvement of Exon 31 alternative splicing in phenotype and severity of epidermolysis bullosa caused by mutations in PLEC1
Daisuke Sawamura, Maki Goto, Kaori Sakai, Hideki Nakamura, James R. McMillan, Masashi Akiyama, Osamu Shirado, Noritaka Oyama, Masataka Satoh, Fumio Kaneko, Toshiaki Takahashi, Hidehiko Konno, Hiroshi Shimizu
JOURNAL OF INVESTIGATIVE DERMATOLOGY, 127, 6, 1537, 1540, Jun. 2007, [Peer-reviewed]
English - Humanization of autoantigen
Wataru Nishie, Daisuke Sawamura, Maki Goto, Kei Ito, Akihiko Shibaki, James R. McMillan, Kaori Sakai, Hideki Nakamura, Edit Olasz, Kim B. Yancey, Masashi Akiyama, Hiroshi Shimizu
NATURE MEDICINE, 13, 3, 378, 383, Mar. 2007, [Peer-reviewed]
English, Scientific journal - ITGB4 missense mutation in a transmembrane domain causes non-lethal variant of junctional epidermolysis bullosa with pyloric atresia.
Abe M, Sawamura D, Goto M, Nakamura H, Nagasaki A, Nomura Y, Kawasaki H, Isogai R, Shimizu H
J Dermatol Sci, 47, 165, 167, 2007, [Peer-reviewed] - Histone deacetylase inhibitors preferentially augment transient transgene expression in human dermal fibroblasts.
Yasukawa K, Sawamura D, Goto M, Nakamura H, Shimizu H
Br J Dermatol, 157, 662, 669, 2007, [Peer-reviewed] - CTACK/CCL27 accelerates skin regeneration via accumulation of bone marrow-derived keratinocytes.
Inokuma D, Abe R, Fujita Y, Sasaki M, Shibaki A, Nakamura H, McMillan JR, Shimizu T, Shimizu H
Stem cells (Dayton, Ohio), 24, 2810, 2816, 12, Dec. 2006, [Peer-reviewed] - Analysis of the COL17A1 in non-Herlitz junctional epidermolysis bullosa and amelogenesis imperfecta.
Nakamura H, Sawamura D, Goto M, Nakamura H, Kida M, Ariga T, Sakiyama Y, Tomizawa K, Mitsui H, Tamaki K, Shimizu H
International journal of molecular medicine, 18, 333, 337, 2, Aug. 2006, [Peer-reviewed] - Epidermolysis bullosa simplex in Japanese and Korean patients: genetic studies in 19 cases.
Yasukawa K, Sawamura D, Goto M, Nakamura H, Jung SY, Kim SC, Shimizu H
The British journal of dermatology, 155, 313, 317, 2, Aug. 2006, [Peer-reviewed] - Colocalization of multiple laminin isoforms predominantly beneath hemidesmosomes in the upper lamina densa of the epidermal basement membrane.
McMillan JR, Akiyama M, Nakamura H, Shimizu H
J Histochem Cytochem, 54, 1, 109, 118, 2006, [Peer-reviewed] - Glycine substitution mutations by different amino acids at the same codon in COL7A1 cause different modes of dystrophic epidermolysis bullosa inheritance.
Sawamura D, Mochitomi Y, Kanzaki T, Nakamura H, Shimizu H
Br J Dermatol, 155, 834, 837, 2006, [Peer-reviewed] - Clinical heterogeneity of 1649delG mutation in the tail domain of keratin 5: a Japanese family with epidermolysis bullosa simplex with mottled pigmentation.
Horiguchi Y, Sawamura D, Mori R, Nakamura H, Takahashi K, Shimizu H
The Journal of investigative dermatology, 125, 83, 85, 1, Jul. 2005, [Peer-reviewed] - Focal palmoplantar callosities in non-Herlitz junctional epidermolysis bullosa
H Mitsui, T Watanabe, M Komine, H Nakamura, H Shimizu, K Tamaki
JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY, 52, 2, 371, 373, Feb. 2005, [Peer-reviewed]
English - Epidermolysis bullosa simplex associated with pyloric atresia is a novel clinical subtype caused by mutations in the plectin gene (PLEC1).
Nakamura H, Sawamura D, Goto M, McMillan JR, Park S, Kono S, Hasegawa S, Paku S, Nakamura T, Ogiso Y, Shimizu H
J Mol Diagn, 7, 28, 35, 2005, [Peer-reviewed] - Genetic studies of 20 Japanese families of dystrophic epidermolysis bullosa.
Sawamura D, Goto M, Yasukawa K, Sato-Matsumura K, Nakamura H, Ito K, Tomita Y, Shimizu H
J Hum Genet, 50, 10, 543, 546, Springer, 2005, [Peer-reviewed]
English, Dystrophic EB (DEB) is clinically characterized by mucocutaneous blistering in response to minor trauma, followed by scarring and nail dystrophy, and is caused by mutations in the COL7A1 gene encoding type VII collagen. DEB is inherited in either an autosomal dominant (DDEB) or recessive (RDEB) fashion. DDEB basically results from a glycine substitution mutation within the collagenous domain on one COL7A1 allele, while a combination of mutations such as premature stop codon, missense, splice-site mutations on both alleles causes RDEB. This study performed mutation analysis in 20 distinct Japanese DEB families (16 RDEB and 4 DDEB). The result demonstrated 30 pathogenic COL7A1 mutations with 16 novel mutations, which included 4 missense, 5 nonsense, 1 deletion, 2 insertion, 1 indel, 3 splice-site mutations. We confirmed that Japanese COL7A1 mutations were basically family specific although 3 mutations 5818delC, 6573+1G>C, E2857X were recurrent based on previous reports. Furthermore, Q2827X mutation found in two unrelated families would be regarded as a candidate recurrent Japanese COL7A1 mutation. The study furthers our understanding of both the clinical and allelic heterogeneity displayed in Japanese DEB patients. - DNA-based prenatal exclusion of bullous congenital ichthyosiform erythroderma at the early stage, 10 to 11 weeks' of pregnancy, in two consequent siblings.
Tsuji-Abe Y, Akiyama M, Nakamura H, Takizawa Y, Sawamura D, Matsunaga K, Suzumori K, Shimizu H
Journal of the American Academy of Dermatology, 51, 6, 1008, 1011, 6, Dec. 2004, [Peer-reviewed]
Scientific journal - Minodronate, a newly developed nitrogen-containing bisphosphonate, suppresses melanoma growth and improves survival in nude mice by blocking vascular endothelial growth factor signaling.
Yamagishi S, Abe R, Inagaki Y, Nakamura K, Sugawara H, Inokuma D, Nakamura H, Shimizu T, Takeuchi M, Yoshimura A, Bucala R, Shimizu H, Imaizumi T
The American journal of pathology, 165, 1865, 1874, 6, Dec. 2004, [Peer-reviewed] - Tyrosinase gene analysis in Japanese patients with oculocutaneous albinism.
Goto M, Sato-Matsumura KC, Sawamura D, Yokota K, Nakamura H, Shimizu H
Journal of dermatological science, 35, 3, 215, 220, 3, Sep. 2004, [Peer-reviewed]
English - Overexpression of pigment epithelium-derived factor decreases angiogenesis and inhibits the growth of human malignant melanoma cells in vivo.
Abe R, Shimizu T, Yamagishi S, Shibaki A, Amano S, Inagaki Y, Watanabe H, Sugawara H, Nakamura H, Takeuchi M, Imaizumi T, Shimizu H
The American journal of pathology, 164, 1225, 1232, 4, Apr. 2004, [Peer-reviewed] - Regulation of human melanoma growth and metastasis by AGE-AGE receptor interactions.
Abe R, Shimizu T, Sugawara H, Watanabe H, Nakamura H, Choei H, Sasaki N, Yamagishi S, Takeuchi M, Shimizu H
The Journal of investigative dermatology, 122, 461, 467, 2, Feb. 2004, [Peer-reviewed] - Linear verrucous haemangioma on the abdomen
H Hayashi, T Shimizu, H Nakamura, H Shimizu
ACTA DERMATO-VENEREOLOGICA, 84, 1, 79, 80, 2004, [Peer-reviewed]
English - Subcutaneous nodules on the buttocks as a manifestation of dialysis-related amyloidosis: a clinicopathological entity?
Shimizu S, Yasui C, Yasukawa K, Nakamura H, Shimizu H, Tsuchiya K
The British journal of dermatology, 149, 400, 404, 2, Aug. 2003, [Peer-reviewed] - Squamous cell carcinoma developing in a 12-year-old boy with nonHallopeau-Siemens recessive dystrophic epidermolysis bullosa
H Kawasaki, D Sawamura, F Iwao, T Kikuchi, H Nakamura, S Okubo, T Matsumura, H Shimizu
BRITISH JOURNAL OF DERMATOLOGY, 148, 5, 1047, 1050, May 2003, [Peer-reviewed]
English, Scientific journal - Toxic epidermal necrolysis and Stevens-Johnson syndrome are induced by soluble Fas ligand.
Abe R, Shimizu T, Shibaki A, Nakamura H, Watanabe H, Shimizu H
The American journal of pathology, 162, 1515, 1520, 5, May 2003, [Peer-reviewed] - A novel insertion mutation in COL7A1 identified in Hallopeau-Siemens recessive dystrophic epidermolysis bullosa.
Sato-Matsumura KC, Sawamura D, Goto M, Nakamura H, Shimizu H
Acta Derm Venereol, 83, 137, 138, 2003, [Peer-reviewed] - A novel A97P amino acid substitution in alpha-galactosidase A leads to a classical Fabry disease with cardiac manifestations
K Kimura, KC Sato-Matsumura, H Nakamura, Y Onodera, K Morita, N Enami, T Shougase, T Ohsaki, M Kato, T Takahashi, Y Yamaguchi, H Shimizu
BRITISH JOURNAL OF DERMATOLOGY, 147, 3, 545, 548, Sep. 2002, [Peer-reviewed]
English, Scientific journal - Histochemical analysis of macrophage migration inhibitory factor in psoriasis vulgaris.
Shimizu T, Nishihira J, Mizue Y, Nakamura H, Abe R, Watanabe H, Ishibashi T, Shimizu H
Histochemistry and cell biology, 118, 251, 257, 3, Sep. 2002, [Peer-reviewed] - High macrophage migration inhibitory factor (MIF) serum levels associated with extended psoriasis.
Shimizu T, Nishihira J, Mizue Y, Nakamura H, Abe R, Watanabe H, Ohkawara A, Shimizu H
The Journal of investigative dermatology, 116, 6, 989, 990, Jun. 2001, [Peer-reviewed] - High expression of macrophage migration inhibitory factor in human melanoma cells and its role in tumor cell growth and angiogenesis
T Shimizu, R Abe, H Nakamura, A Ohkawara, M Suzuki, J Nishihira
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 264, 3, 751, 758, Nov. 1999, [Peer-reviewed]
English, Scientific journal
Other Activities and Achievements
- Two families of nail-patella syndrome with novel splice site mutations in the LMX1B gene
S. Shinkuma, H. Nakamura, S. Takashima, T. Nomura, Y. Fujita, S. Hasegawa, K. Matsumura, H. Shimizu, R. Abe, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 137, 10, S231, S231, Oct. 2017
English, Summary international conference - 皮膚基底膜構築における17型コラーゲン細胞外領域切断の生理機能の解析
西村 真智子, 西江 渉, 新熊 悟, 夏賀 健, 中村 秀樹, 清水 宏, 白藤 宜紀, 岩月 啓氏, 澤村 大輔, 日本皮膚科学会雑誌, 127, 9, 2111, 2111, Aug. 2017
(公社)日本皮膚科学会, Japanese - A case of recessive dystrophic epidermolysis bullosa with a novel c.6885_6898del14 mutation
S. Shinkukma, T. Masunaga, S. Miyawaki, S. Takashima, T. Nomura, Y. Fujita, H. Nakamura, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 137, 5, S87, S87, May 2017
English, Summary international conference - 新規ケラチン1遺伝子変異によるichthyosis with confettiの1家系
鈴木 翔多朗, 乃村 俊史, 宮内 俊成, 竹田 真依, 中村 秀樹, 新熊 悟, 藤田 靖幸, 清水 宏, 秋山 真志, 角化症研究会記録集, 31, 12, 17, Mar. 2017
角化症研究会事務局, Japanese - 重症汎発型単純型表皮水疱症の家族例
須貝 達朗, 新熊 悟, 高島 翔太, 乃村 俊史, 藤田 靖幸, 中村 秀樹, 清水 宏, 稲福 和宏, 日本皮膚科学会雑誌, 127, 2, 221, 221, Feb. 2017
(公社)日本皮膚科学会, Japanese - Defective binding of plectin and collagen XVII leads to epidermolysis bullosa simplex
K. Natsuga, W. Nishie, M. Nishimura, S. Shinkuma, M. Watanabe, K. Izumi, H. Nakamura, Y. Hirako, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 136, 9, S186, S186, Sep. 2016
English, Summary international conference - Keratinocyte death in severe cutaneous adverse drug reactions is mediated by necroptosis pathway of annexin A1/FPR1 interaction
N. Saito, T. Yanagi, S. Shinkuma, A. Suto, Y. Fujita, S. Suzuki, T. Nomura, H. Nakamura, H. Shimizu, R. Abe, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 134, S3, S3, May 2014
English, Summary international conference - Loss of ELOVL1 enzyme causes lethal skin barrier disruption in mice
S. Suzuki, T. Nomura, T. Sassa, Y. Ohno, H. Nakamura, M. Akiyama, A. Kihara, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 134, 4, 1177, 1177, Apr. 2014
English, Summary international conference - A CASE OF RECESSIVE DYSTROPHIC EPIDERMOLYSIS BULLOSA WITH LONG-TERM FOLLOW-UP OF CULTURED EPIDERMAL AUTOGRAFT
Y. Fujita, S. Shinkuma, D. Sawamura, H. Kawasaki, H. Nakamura, M. Inoie, W. Nishie, H. Shimizu, WOUND REPAIR AND REGENERATION, 22, 1, A18, A18, Jan. 2014
English, Summary international conference - The mitochondrial fusion proteins Mfn1 and Mfn2 are involved in keratinization
A. Suto, R. Abe, N. Saito, S. Suzuki, Y. Fujita, T. Nomura, H. Nakamura, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 133, S109, S109, May 2013
English, Summary international conference - A novel necroptosis pathway of annexin A1/FPR1 interaction in severe cutaneous adverse drug reactions
N. Saito, H. Qiao, T. Yanagi, S. Shinkuma, A. Suto, Y. Fujita, S. Suzuki, T. Nomura, H. Nakamura, H. Shimizu, R. Abe, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 133, S28, S28, May 2013
English, Summary international conference - Bullous pemphigoid autoantibodies induce skin fragility in neonatal mice in a complement independent manner
T. Sasaoka, H. Ujiie, W. Nishie, A. Shibaki, H. Nakamura, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 133, S29, S29, May 2013
English, Summary international conference - 31-year follow-up of Kindler syndrome with severe intestinal involvement
S. Shimizu, W. Nishie, K. Tsuchiya, D. Inokuma, H. Koguchi, K. Kikuchi, S. Motoya, H. Nakamura, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 133, S129, S129, May 2013
English, Summary international conference - Essential role of collagen XVII in basement membrane formation and keratinocyte migration
M. Nishimura, W. Nishie, Y. Shirafuji, S. Shinkuma, K. Natsuga, H. Nakamura, D. Sawamura, K. Iwatsuki, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 133, S151, S151, May 2013
English, Summary international conference - アトピー性皮膚炎および先天性表皮水疱症として経過観察されていたKindler症候群の2例
神谷 浩二, 白藤 宜紀, 青山 裕美, 岩月 啓氏, 新熊 悟, 夏賀 健, 中村 秀樹, 西江 渉, 清水 宏, 日本皮膚科学会雑誌, 123, 4, 456, 456, Apr. 2013
(公社)日本皮膚科学会, Japanese - ケラチン5のコイル1A領域N末端部にミスセンス変異を生じた単純型表皮水疱症
新熊 悟, 西江 渉, 夏賀 健, 氏家 英之, 中村 秀樹, 清水 宏, Jacyk Witold, 秋山 真志, 日本皮膚科学会雑誌, 123, 4, 461, 461, Apr. 2013
(公社)日本皮膚科学会, Japanese - 劣性栄養障害型表皮水疱症患者における遺伝子型-表現型の相互関係の解析
新熊 悟, 西江 渉, 夏賀 健, 伊藤 圭, 中村 秀樹, 清水 宏, 日本皮膚科学会雑誌, 122, 2, 381, 381, Feb. 2012
(公社)日本皮膚科学会, Japanese - 【橋本公二コネクション-薬疹・水疱症・そして皮膚科医人生-】 (Part2)水疱症関連 (case 07)非Herlitz型接合部型表皮水疱症
松本 圭子, 橋本 公二, 薬師寺 直喜, 西江 渉, 夏賀 健, 中村 秀樹, 清水 宏, Visual Dermatology, 11, 2, 165, 167, Jan. 2012
(株)学研メディカル秀潤社, Japanese - プレクチンの発現様式の違いによって2種類の表皮水疱症の臨床型が決定される
夏賀 健, 西江 渉, 秋山 志, 新熊 悟, 中村 秀樹, 長崎 暁理, 澤村 大輔, 清水 宏, Has Cristina, Bruckner-Tuderman Leena, 大内 健嗣, 石河 晃, 平子 善章, 尾張部 克志, 日本皮膚科学会雑誌, 122, 1, 57, 57, Jan. 2012
(公社)日本皮膚科学会, Japanese - Distinctive anchoring fibril structures produced by different COL7A1 mutations
S. Shinkuma, W. Nishie, K. Natsuga, H. Nakamura, K. Ito, M. Akiyama, H. Shimizu, EXPERIMENTAL DERMATOLOGY, 19, 11, 1032, 1032, Nov. 2010
English, Summary international conference - アトピー性皮膚炎との鑑別を要したKindler症候群の一例
白藤 宜紀, 青山 裕美, 岩月 啓氏, 梅村 茂夫, 新熊 悟, 夏賀 健, 中村 秀樹, 西江 渉, 清水 宏, Journal of Environmental Dermatology and Cutaneous Allergology, 4, 5, 437, 437, Nov. 2010
(一社)日本皮膚アレルギー・接触皮膚炎学会, Japanese - New insight into genotype-phenotype correlation in recessive dystrophic epidermolysis bullosa with a range of COL7A1 missense mutations.
Satoru Shinkuma, Wataru Nishie, Ken Natsuga, Hideki Nakamura, Kei Ito, Hiroshi Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 130, S82, S82, Sep. 2010
English, Summary international conference - Erratum: Bone marrow transplantation restores epidermal basement membrane protein expression and rescues epidermolysis bullosa model mice (Proceedings of the National Academy of Sciences of the United States of America (2010) 107, (14345-14350) DOI: 10.10
Yasuyuki Fujita, Riichiro Abe, Daisuke Inokuma, Mikako Sasaki, Daichi Hoshina, Ken Natsuga, Wataru Nishie, James R. McMillan, Hideki Nakamura, Tadamichi Shimizu, Masashi Akiyama, Daisuke Sawamura, Hiroshi Shimizu, Proceedings of the National Academy of Sciences of the United States of America, 107, 14514, 10 Aug. 2010 - Bone marrow transplantation restores epidermal basement membrane protein expression and rescues epidermolysis bullosa model mice (vol 107, pg 32, 2010)
Yasuyuki Fujita, Riichiro Abe, Daisuke Inokuma, Mikako Sasaki, Daichi Hoshina, Ken Natsuga, Wataru Nishie, James R. McMillan, Hideki Nakamura, Tadamichi Shimizu, Masashi Akiyama, Daisuke Sawamura, Hiroshi Shimizu, PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 107, 32, 14514, 14514, Aug. 2010
English, Others - Both hematopoietic and mesenchymal stem cells contribute to the production of basement membrane protein in bone marrow transplantation treatment model mouse
Y. Fujita, D. Inokuma, W. Nishie, H. Nakamura, M. Akiyama, D. Sawamura, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 129, S44, S44, Sep. 2009
English, Summary international conference - Novel therapeutic strategy for bullous pemphigoid using recombinant monoclonal fab antibody against NC16A domain of collagen XVII
G. Wang, A. Shibaki, H. Ujiie, W. Nishie, Y. Tateishi, K. Kikuchi, Q. Li, J. R. McMilan, H. Morioka, D. Sawamura, H. Nakamura, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 128, S12, S12, Apr. 2008
English, Summary international conference - Development of an active disease model for bullous pemphigoid
H. Ujiie, A. Shibaki, W. Nishie, G. Wang, Y. Tateishi, H. Nakamura, M. Akiyama, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 128, S10, S10, Apr. 2008
English, Summary international conference - Bone marrow transplantation restores deficient epidermal basement membrane protein and improves the clinical phenotype in epidermolysis bullosa model mice
Y. Fujita, R. Abe, D. Inokuma, M. Sasaki, D. Hoshina, W. Nishie, J. R. McMillan, H. Nakamura, T. Shimizu, D. Sawamura, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 128, S114, S114, Apr. 2008
English, Summary international conference - Rescue of lethal dystrophic epidermolysis bullosa model mice by corrective COL7A1 gene transfer
K. Ito, D. Sawamura, W. Nishie, H. Nakamura, M. Goto, A. Shibaki, J. Uitto, M. Akiyama, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 127, S78, S78, Oct. 2007
English, Summary international conference - ムラージュ (ロウ製皮膚病模型)
中村 秀樹, 清水 宏, 北海道醫學雜誌 = Acta medica Hokkaidonensia, 82, 4, 261, 266, 01 Jul. 2007
Japanese - Production of a murine model of epidermolysis bullosa (EB)
W. Nishie, D. Sawamura, M. Coto, K. Ito, A. Shibaki, J. R. McMillan, K. Sakai, H. Nakamura, E. Olasz, K. B. Yancey, M. Akiyama, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 126, 12, 2744, 2744, Dec. 2006
English, Summary international conference - Genetic studies of 20 Japanese families of dystrophic epidermolysis bullosa (vol 50, pg 543, 2005)
Daisuke Sawamura, Maki Goto, Kana Yasukawa, Kazuko Sato-Matsumura, Hideki Nakamura, Kei Ito, Hiroyuki Nakamura, Yuki Tomita, Hiroshi Shimizu, JOURNAL OF HUMAN GENETICS, 51, 9, 839, 839, Sep. 2006
English, Others - Possible involvement of exon 31 alternative splicing in marked different clinical manifestation caused by mutations in the plectin gene (PLEC1)
D. Sawamura, M. Goto, H. Nakamura, N. Oyama, M. Satoh, F. Kaneko, T. Takahashi, H. Konno, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 126, 24, 24, Aug. 2006
English, Summary international conference - Collagen XVII/BP180 knockout mouse and its transgenic rescue using human collagen XVII cDNA
W. Nishie, D. Sawamura, M. Goto, A. Shibaki, J. R. McMillan, H. Nakamura, E. Olasz, K. B. Yancey, M. Akiyama, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 126, 21, 21, Aug. 2006
English, Summary international conference - COL7A1 mutation G2037E causes epidermal retention of type VII collagen
SAWAMURA Daisuke, SATO MATSUMURA Kazuko, SHIBATA Satoko, TASHIRO Akari, FURUE Masutaka, GOTO Maki, SAKAI Kaori, AKIYAMA Masashi, NAKAMURA Hideki, SHIMIZU Hiroshi, Journal of Human Genetics, 51, 5, 418, 423, 2006
COL7A1 glycine substitution (GS) mutations result in dominant and recessive dystrophic epidermolysis bullosa (DDEB and RDEB). Here, we report a DDEB family in which a female proband showed retention of type VII collagen inepidermal keratinocytes. Mutational analysis detected a GS mutation; G2037E in the proband and her affected father. To demonstrate the direct association of G2037E and type VII collagen retention, we have introduced this mutated COL7A1 gene into cultured keratinocytes using retroviral methods. This mutation was dominant, so we transferred a 1:1 mixture of wild type (unaffected) and G2037E mutated COL7A1 together, in addition to the unaffected gene or the mutated gene alone. An increase in type VII collagen cytoplasmic staining in the G2037E/wild transfectant cell samples compared with the control/wild type cells. The G2037E (alone) transfected cells showed even stronger intracellular collagen VII staining than the G2037E/wild transfection sample. These results demonstrate that the G2037E COL7A1 mutation leads to increased epidermal retention of type VII collagen in vivo, and also suggests that homozygotes carrying this dominant GS mutation may show more severe phenotypes than heterozygotes. This study furthers our understanding of GS COL7A1 mutations in DEB., Springer, English - Tight junction proteins show developmentally restricted expression patterns during human fetal skin ontogeny
McMillan, JR, M Akiyama, H Nakamura, H Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 121, 4, 944, 944, Oct. 2003
English, Summary international conference - Subcutaneous nodules on the buttocks as a manifestation of dialysis-related amyloidosis: Ultrastructural and immunohistochemical study
S Shimizu, C Yasui, K Yasukawa, H Nakamura, H Shimizu, K Tsuchiya, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 121, 4, 945, 945, Oct. 2003
English, Summary international conference - advanced glycation end products(AGEs)及びAGE受容体のメラノーマにおける増殖能,転移能への関与
阿部 理一郎, 清水 忠道, 渡辺 宏数, 中村 秀樹, 長栄 洋, 佐々木 信幸, 山岸 昌一, 竹内 正義, 清水 宏, 日本皮膚科学会雑誌, 113, 10, 1567, 1568, Sep. 2003
(公社)日本皮膚科学会, Japanese - Regulation of human malignant melanoma growth and metastasis by AGE-AGE receptor interaction
R Abe, T Shimizu, H Watanabe, H Nakamura, H Choei, N Sasaki, S Yamagishi, M Takeuchi, H Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 119, 1, 216, 216, Jul. 2002
English, Summary international conference - Dominant and recessive compound heterozygous mutations in Epidermolysis Bullosa simplex demonstrate the role of the stutter region in keratin intermediate filament assembly
YASUKAWA K, SAWAMURA D, MCMILLAN J R, NAKAMURA H, SHIMIZU H, J Biol Chem, 277, 26, 23670, 23674, 2002 - Histochemical analysis of macrophage migration inhibitory factor (MIF) in psoriasis vulgaris
T Shimizu, J Nishihira, Y Mizue, H Nakamura, P Abe, H Watanabe, H Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 117, 3, 808, 808, Sep. 2001
English, Summary international conference
Research Themes
- Development of a novel therapeutic strategy for angiosarcoma through drug screening
Grants-in-Aid for Scientific Research
01 Apr. 2023 - 31 Mar. 2026
中村 秀樹, 柳 輝希, 氏家 英之
Japan Society for the Promotion of Science, Grant-in-Aid for Scientific Research (C), Hokkaido University, 23K07757 - Genome-wide screening of the key molecules for metastasizing collagen VII-deficient squamous cell carcinoma.
Grants-in-Aid for Scientific Research
01 Apr. 2019 - 31 Mar. 2022
Nakamura Hideki
The aim of this study was to elucidate the metastatic mechanism of squamous cell carcinoma (SCC) arising in patients with dystrophic epidermolysis bullosa (DEB) caused by mutations in type 7 collagen (COL7) gene. DEB patient model keratinocytes with a comprehensive gene-editing did not develop metastasis when transplanted subcutaneously in mice, but the subcutaneous tumors of DEB patient model showed an overall irregular structure, surrounding fibrosis, and hypervascularization compared to subcutaneous tumors of normal keratinocytes. These results suggest that subcutaneous tumors in DEB patient model induce changes in the surrounding tissue environment via abnormal construction of the basement membrane, and that these changes may contribute to the metastatic mechanism of SCC in DEB patients.
Japan Society for the Promotion of Science, Grant-in-Aid for Scientific Research (C), Hokkaido University, 19K08764 - Establishment of cicatricial alopecia animal models
Grants-in-Aid for Scientific Research
01 Apr. 2016 - 31 Mar. 2019
Nakamura Hideki
Cicatricial alopecia is a type of hair loss characterized by loss of hair follicles and surrounding fibrosis. The aim of this study was to elucidate the mechanism of cicatricial alopecia by analyzing the hair follicles in the blistered skin of mice in detail. A major finding was a delay in hair follicle development/cycling at the re-epithelialized wounds after blistering. At the same time, infiltration of inflammatory cells was poor at the site where several layers of epithelization were completed, and there was no difference compared to the intact site. Taken together, these results indicate that the early stage of cicatricial alopecia involves less inflammation, with the proliferation of epithelial cells required for hair follicle growth directed toward re-epithelialization.
Japan Society for the Promotion of Science, Grant-in-Aid for Scientific Research (C), Hokkaido University, 16K10120 - Elucidating roles of plasmin in blister formation of bullous pemphigoid
Grants-in-Aid for Scientific Research
01 Apr. 2013 - 31 Mar. 2016
Nakamura Hideki, NISHIE Wataru, NATSUGA Ken, SHIMIZU Hiroshi
Bullous pemphigoid (BP) is an autoimmune blistering disease. The aim of this study is to address the pathogenic roles of plasmin, a protease present in blister fluids of BP, which is known to cleave COL17. We first identified cleavage sites of COL17 by plasmin, and based on this data, cleavage site-specific antibodies were generated. The antibodies revealed that COL17 is actually cleaved by plasmin in lesional skin of certain numbers of BP patients.
Japan Society for the Promotion of Science, Grant-in-Aid for Scientific Research (C), Hokkaido University, 25461661 - Investigation of hematopoietic stem cell transplantation for epidermolysis bullosa
Grants-in-Aid for Scientific Research
01 Apr. 2012 - 31 Mar. 2015
SHIMIZU Hiroshi, ABE Riichiro, NISHIE Wataru, FUJITA Yasuyuki, AKIYAMA Masashi, KOJIMA Seiji, NAKAMURA Hideki
Epidermolysis bullosa (EB) is a group of genodermatoses that cause blister formations from the congenital abnormality of anchor proteins between the epidermis and the dermis. There have been several strategies for the treatment of EB, and so far, cell therapies are the most promising approach because of the potential of systemic effects. We have proved that stem cell therapies, including bone marrow transplantation, hematopoietic stem cell transplantation, can ameliorate the phenotype and survival prognosis in the junctional EB model mice that lack type XVII collagen (Col17). In this study we explore more efficient approaches of stem cell therapies for EB, including intramedullary transplantation, mesenchymal stromal/stem cell infusion, and investigate factors in association with transdifferentiation from bone marrow-derived stem cells into keratinocytes.
Japan Society for the Promotion of Science, Grant-in-Aid for Scientific Research (A), Hokkaido University, 24249062 - Objective Evaluation Method of Genetic Treatment for Epidermolysis bullosa
Grants-in-Aid for Scientific Research
2007 - 2008
NAKAMURA Hideki, SHIMIZU Hiroshi, NISHIE Wataru
non-Herlitz接合部型表皮水疱症レスキューマウスを詳細に解析し、遺伝子導入治療の客観的評価法を検討した。レスキューマウスに生じる臨床効果の差異は超微細構造学的構造変化と蛋白発現によっても確認することができた。これらの差異が生じる要因として遺伝子導入状態の違いを確認することができたが、この違いを明らかにするためには導入されたgenomic DNAを確認するだけでは不十分であった。臨床効果を分岐する遺伝子導入状態の分岐点を明らかにするためにはrealtime PCRによるmRNAの定量、Fluorescence in-situ Hybridization法による遺伝子導入部位の確認が必要である。
Japan Society for the Promotion of Science, Grant-in-Aid for Scientific Research (C), Hokkaido University, 19591291
