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＜教授・略歴＞

昭和５６年　京都府立医科大学卒
昭和６０年　京都府立医科大学大学院修了、医学博士
昭和６０年　福井医科大学皮膚科学講座文部教官助手
昭和６１年　大阪大学細胞工学センター遺伝子情報研究部門研究生
昭和６２年　京都府立医科大学皮膚科学教室助手　
平成　３年　京都府立医科大学皮膚科学教室講師
平成１４年　三重大学医学部皮膚科学講座文部科学教官助教授
平成１５年１２月より　現職

現在の研究テーマ：皮膚疾患の分子病態解析と、それに基づく新しい診断・治療法の開発

＜主な研究業績：山西清文＞

Tominaga C, Yamamoto M, Ima Yi, Yamanishi K. A case of old age-onset generalized pustular psoriasis with a deficiency of IL-36RN (DITRA) treated by granulocyte and monocyte apheresis. Case Reports in Dermatology (in press)

Suga Y, Tsuda T, Nagai M, Sakaguchi Y, Jitsukawa O,Yamamoto M, Hitomi K, Yamanishi K. Lamellar ichthyosis with pseudoexon activation in the transglutaminase 1 gene. J Dermatol (in press)

Inoue Y, Yamamoto M, Sakaguchi Y, Jitsukawa O, Hayano K, Yamane M, Sakamoto M, Yamanishi K. ABCA12-deficient Congenital ichthyosiform erythroderma in a boy with an intellectual developmental delay. Acta Derm Venereol (in press)

Nagai M, Nagai H, Tominaga C, Sakaguchi Y, Jitsukawa O, Ohgo N, Nishigori C, Yamanishi K. Localised dominant dystrophic epidermolysis bullosa with a novel de novo mutation in COL7A1 diagnosed by next-generation sequencing. Acta Derm Venereol (in press)

Kawasaki K, Yamanishi K, Yamada H.Age-related morphometric changes of inner structures of the skin assessed by in vivo reflectance confocal microscopy.Int J. Dermatol (in press)

Washio K, Fukunaga A, Terai M, Hitomi K, Yamanishi K, Nishigori C. Hypohidrosis plays a crucial role in the vicious circle of bathing suit ichthyosis: a case with summer exacerbation. Acta Derm Venereol 94:349-350, 2014

Imai Y, Yasuda K, Sakaguchi Y, Futatsugi-Yumikura S, Yoshimoto T, Nakanishi K, Yamanishi K. Immediate-type contact hypersensitivity is reduced in interleukin-33 knockout mice. J Dermatol Sci 74:159-161, 2014

Yoshimi N, Imai Y, Kakuno A, Tsubura A, Yamanishi K. Epithelial keratin and filaggrin expression in seborrhoeic keratosis - Evaluation based on histopathological classification.Int J. Dermatol 53:707-713, 2014

Imai Y, Yasuda K, Sakaguchi Y, Haneda T, Mizutani H, Yoshimoto T, Nakanishi K, Yamanishi K.Skin-specific expression of IL-33 activates group 2 innate lymphoid cells and elicits atopic dermatitis-like inflammation in mice.Proc Natl Acad Sci USA 110:13921-6, 2013

Itoh M, Tatsukawa H, Eun-Seo L,Yamanishi K, Kojima S, Hitomi K.Variations in Both TG1 and TG2 isozyme-specific in situ activities and protein expressions during mouse embryonic development. J Histochem Cytochem. 61:793-801, 2013

Yamamoto M, Imai Y, Sakaguchi Y, Haneda T, Yamanishi K. Serum cytokines correlated with the disease severity of generalized pustular psoriasis. Dis Markers 34:153-161, 2013

Otani N, Yamanishi K, Sakaguchi Y, Imai Y, Shima M, Okuno T. Varicella-zoster virus-specific cell-mediated immunity in subjects with herpes zoster. J Immunol Methods 30:53-55,2012

Nakagawa N,Yamamoto M, Imai Y, Sakaguchi Y, Takizawa T, Noboru O,Yagi N,Hatta I, Hitomi K, Takizawa T, Tsuda T, Matsuki M, Takeda J, Yamanishi K. Knocking-in the R142C mutation in transglutaminase 1 disrupts the stratum corneum barrier and postnatal survival of mice. J Dermatol Sci 65:196-206,2012

Yamamoto M, Sakaguchi Y, Itoh M, Nakagawa N, Fukunaga A, Hitomi K, Yamanishi K. Bathing suit ichthyosis with summer exacerbation: A temperature-sensitive case.Br J Dermatol 166:672-674,2012

Imai Y, Tsuda T, Aochi S, Yumikura S, Sakaguchi Y, Nakagawa N, Iwatsuki K, Yamanishi K. YKL-40 (Chitinase 3-Like-1) as a biomarker for psoriasis vulgaris and pustular psoriasis.J Dermatol Sci 64:75-77,2011

Aochi S, Tsuji K, Sakaguchi M, Huh N, Tsuda T, Yamanishi K, Komine M, Iwatsuki K. Markedly elevated serum levels of calcium-binding S100A8/A9 proteins in psoriatic arthritis are due to activated monocytes/macrophages J Am Acad Dermatol 64:879-887,2011

Itoh M, Kawamoto T, Tatsukawa H, Kojima S, Yamanishi K, Hitomi K.In Situ Detection of Active Transglutaminases for Keratinocyte-type (TGase 1) and Tissue-type (TGase 2) Using Fluorescence-labeled Highly Reactive Substrate Peptides. J Histochem Cytochem. 59:180-187,2011

Omoto Y, Yamanaka K, Tokime K, Kitano S, Kakeda M, Akeda T, Kurokawa I, Gabazza EC, Tsutsui H, Katayama N, Yamanishi K, Nakanishi K, Mizutani H. Granzyme B is a novel interleukin-18 converting enzyme J Dermatol Sci.59: 129-35, 2010

Tarutani M, Imai Y, Yasuda K, Tsutsui H, Nakanishi K, Yamanishi K. Neutrophil-dominant psoriasis-like skin inflammation induced by epidermal-specific expression of Raf in mice. J Dermatol Sci. 58:28-35, 2010

Ishikawa C, Tsuda T, Konishi H, Nakagawa N, Yamanishi K. Tetracyclines modulate protease-activated receptor 2-mediated proinflammatory reactions in epidermal keratinocytes. Antimicrob Agents Chemother, 53:1760-1765,2009

Fujimoto T, Tsuda T, Yamamoto M, Tarutani M, Natsuaki M, Minami S, Ito T, Kozuka T, Yamanishi K. Cutaneous malignant fibrous histiocytoma (undifferentiated pleomorphic sarcoma) arising in a chronic scalp ulcer of a patient with non-bullous congenital ichthyosiform erythroderma. J Eur Acad Dermatol Venereol, 23:202-203, 2009

Tsujimura A, Matsuki M, Takao K, Yamanishi K, Miyakawa T, Hashimoto-Gotoh T. Mice lacking the kf-1 gene exhibit increased anxiety- but not despair-like behavior. Frontiers Behavioral Neurosci, 2:1-11, 2008

Sugimura Y, Hosono M, Kitamura M, Tsuda T, Yamanishi K, Maki M, Hitomi K. Identification of preferred substrate sequences for transglutaminase 1-development of a novel peptide that can efficiently detect cross-linking enzyme activity in the skin. FEBS J, 275:5667-5677,2008

Tsuda T, Ishikawa C, Nakagawa N, Konishi H, Tarutani M, Matsuki M, Yamanishi K. A novel point mutation of keratin 17 (KRT17) in a Japanese family with pachyonychia congenita type 2: An RNA-based genetic analysis using a single hair bulb. Br J Dermatol, 159:730-732,2008

Miyata A, Natsuaki M, Yamanishi K. Staphylococcal enterotoxin B enhances a flare-up reaction of murine contact hypersensitivity through up-regulation of interferon-gamma. Exp Dermatol, 17:843-848, 2008

Tsuda T, Ishikawa C, Konishi H, Hayashi Y, Nakagawa N, Matsuki M, Mizutani H, Yamanishi K. The effect of 14-membered ring macrolides on the production of interleukin-8 mediated by protease-activated receptor 2 in human keratinocytes. Antimicrob Agents Chemother, 52:1538-1541, 2008

Omoto Y, Tokime K,Yamanaka K, Habe K, Morioka T, Kurokawa I, Tsutsui H, Yamanishi K, Nakanishi, K, Mizutani H. Human mast cell chymase cleaves pro-IL-18 and generates a novel and biologically active IL-18 fragment.
J Immunol, 177:8315-8319, 2006

Terada M, Tsutsui H, Yasutomo I, Yasuda K, Mizutani H, Yamanishi K, Kubo M, Matsui K, Sano H, Nakanishi K. Contribution of interleukin-18 to atopic dermatitis-like skin inflammation induced by Staphylococcus aureus product in mice. Proc Natl Acad Sci USA, 103:8816-8821, 2006

Matsuno K, Yamada H, Iwata K, Jin D, Katsuyama M,　Matsuki M, Takai S, Yamanishi K, Miyazaki M,Hiroaki Matsubara, Yabe-Nishimura C. Nox1 Is Involved in Angiotensin II-mediated Hypertension − A Study in Nox1-deficient mice, Circulation 112: 2677-2685, 2005

Kuramoto N, Takizawa, T, Takizawa, T, Matsuki M, Morioka H, Robinson JM, Yamanishi K. Development of ichthyosiform skin compensates for defective permeability barrier function in mice lacking transglutaminase 1.
J Clin Invest 109: 243-250, 2002

Shen CS, Tabata K, Matsuki M, Goto T, Yokochi T, Yamanishi K. Premature apoptosis of keratinocytes and the dysregulation of keratinization in porokeratosis. Br J Dermatol 147: 498-502, 2002

Sayama K, Hanakawa Y, Shirakata Y, Yamasaki K, Sawada Y, Sun L, Yamanishi K, Ichijo H, Hashimoto K. Apoptosis signal regulating kinase 1 (ASK1) is an intracellular inducer of keratinocyte differentiation. J Biol Chem 276: 999-1004, 2001

Inada R, Matsuki M, Yamada K, Morishima Y, Shen S-C, Kuramoto N, Yasuno H, Takahashi K, Miyachi Y, and Yamanishi K. Facilitated wound healing by activation of the transglutaminase 1 gene. Am J Pathol 157: 1875-1882, 2000

Hiiragi T，Sasaki H，Nagafuchi A，Sabe H，Shen SC，Matsuki M，Yamanishi K, Tsukita S. Transglutaminase type 1 and its cross-linking activity are concentrated at adherens junctions in simple epithelial cells. J Biol Chem 274:34148-34154, 1999

Nishida K，Yamanishi K，Yamada K，Dota A，Kawasaki S，Quantock AJ, Kinoshita S. Epithelial hyperproliferation and transglutaminase 1 gene expression in Stevens-Johnson syndrome conjunctiva. Am J Pathol 154:331-336, 1999

Matsuki M, Yamashita F, Ishida-Yamamoto A, Yamada K, Kinoshita C, Fushiki S, Ueda E, Morishima Y, Tabata K, Yasuno H, Hashida M, Iizuka H, Ikawa M, Okabe M, Kondoh G, Kinoshita T, Takeda J, Yamanishi K. Defective stratum corneum and early neonatal death in mice lacking the gene for transglutaminase 1 (keratinocyte transglutaminase). Proc Natl Acad Sci USA 95 :1044-1049, 1998

Takahashi K, Yan B, Yamanishi K, Imamura S, Coulombe PA. The two functional keratin 6 genes of mouse are differentially regulated and evolved independently from their human orthologs. Genomics 53:170-183, 1998

Kawabe S, Ikuta T, Ohba M, Chida K, Ueda E, Yamanishi K, Kuroki T. Cholesterol sulfate activates transcription of transglutaminase 1 gene in normal human keratinocytes. J Invest Dermatol 111 :1098-1102, 1998

Yamada K, Matsuki M, Morishima Y, Ueda E, Tabata K, Yasuno H, Suzuki M, Yamanishi K. Activation of the human transglutaminase 1 promoter in transgenic mice: Terminal differentiation-specific expression of theTGM1-lacZ transgene in keratinized stratified squamous epithelia. Hum Mol Genet 6 :2223-2231, 1997

Ueda E，Ohno S，Kuroki T，Livneh E，Yamada K，Yamanishi K, Yasuno H. The eta isoform of protein kinase C mediates transcriptional activation of the human transglutaminase 1 gene. J Biol Chem 271:9790-9794, 1996

Morishima Y，Ariyama T，Yamanishi K，Abe T，Ueda E，Yasuno H,  Inazawa J. Chromosomal loci of 50 human keratinocyte cDNAs assigned by fluorescence in situ hybridization. Genomics 28:273-279, 1995

Konishi K，Morishima Y，Ueda E，Kibe Y，Nonomura K，Yamanishi K, Yasuno H. Cataloging of the genes expressed in human keratinocytes: Analysis of  607 randomly isolated cDNA sequences． Biochem Biophys Res Commun 202:976-983, 1994

Yamanishi K，Matsuki M，Konishi K, Yasuno H. A novel mutation of Leu122 to Phe at a highly conserved hydrophobic residue in the helix initiation motif of keratin 14 in epidermolysis bullosa simplex. Hum Mol Genet 3:1171-1172,1994

Nonomura K, Yamanishi K, Yasuno H，Nara K, Hirose S. Up-regulation of elafin/SKALP gene expression in psoriatic epidermis. J Invest Dermatol 103:88-91, 1994

Nonomura K，Yamanishi K，Hosokawa Y，Doi H，Hirano J，Fukushima S, Yasuno H Localization of transglutaminase 1 mRNA in normal and psoriatic epidermis by non-radioactive in situ hybridization. Br J Dermatol 128:23-28, 1993

Yamanishi K，Inazawa J，Liew FM， Nonomura K，Ariyama T，Yasuno H，Abe T，Doi H，Hirano J, Fukushima S. Structure of the gene for human transglutaminase 1. J Biol Chem 267:17858-17863, 1992

Yamanishi K， Liew FM， Konishi K，Yasuno H，Doi H，Hirano J, Fukushima S. Molecular cloning of human epidermal transglutaminase cDNA from keratinocytes in culture. Biochem Biophys Res Commun 175:906-913,1991

Fujita T，Shibuya H，Ohashi T，Hotta H，Yamanishi K, Taniguchi T.  Interferon-β gene regulation: Tandemly repeated sequence of a synthetic 6 bp oligomer function as a virus-inducible enhancer.Cell 49:357-367, 1987

Fujita T，Shibuya H，Ohashi T， Yamanishi K,　Taniguchi T. Regulation of human interleukin-2 gene：Functional DNA sequence in the 5' flanking region for the gene expression in activated T lymphocytes. Cell 46:401-407,1986