Pathological mechanisms of keratinization abnormalities
Project/Area Number |
19591294
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Research Category |
Grant-in-Aid for Scientific Research (C)
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Allocation Type | Single-year Grants |
Section | 一般 |
Research Field |
Dermatology
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Research Institution | Asahikawa Medical College |
Principal Investigator |
YAMAMOTO Akemi Asahikawa Medical College, 医学部, 准教授 (30241441)
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Project Period (FY) |
2007 – 2010
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Project Status |
Completed (Fiscal Year 2010)
|
Budget Amount *help |
¥4,290,000 (Direct Cost: ¥3,300,000、Indirect Cost: ¥990,000)
Fiscal Year 2010: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2009: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2008: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2007: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
|
Keywords | 皮膚病理学 / 角化細胞 / 魚鱗癬 / 角化症 / 角化 / 遺伝子異常 / 免疫電顕法 / モデル動物 / 超微構造 / プロテアーゼ / 皮膚バリア |
Research Abstract |
We have studied mechanisms of normal keratinization and abnormal keratinization. We have found evidences to suggest that tight junctions are involved in spatially distinct degradation of corneodesmosomes and in lamellar granule transportation and secretion. We have revealed that abnormal transportation and/or secretion of lamellar granules could result in ichthyosis in two genetic diseases, CEDNIK syndrome and ARC syndrome. We found that corneodesmosin in crucial for corneocyte adhesion. We have found evidences to suggest that Rab11 may be involved in lamellar granule transportation.
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Report
(6 results)
Research Products
(83 results)
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[Journal Article] RIN2 deficiency results in macrocephaly, alopecia, cutis laxa, and scoliosis: MACS syndrome.2009
Author(s)
Basel-Vanagaite L, Sarig O, Hershkovitz D, Fuchs-Telem D, Rapaport D, Gat A, Isman G, Shirazi I, Shohat M, Enk CD, Birk E, Kohlhase J, Matysiak-Scholze U, Maya I, Knopf C, Peffekoven A, Hennies HC, Bergman R, Horowitz M, Ishida-Yamamoto A, Sprecher E.
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Journal Title
Am J Hum Genet 85
Pages: 254-63
Related Report
Peer Reviewed
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[Journal Article] Alopecia, neurological defects, and endocrinopathy syndrome caused by decreased expression of RBM28, a nucleolar protein associated with ribosome biogenesis.2008
Author(s)
Nousbeck J, Spiegel R, Ishida-Yamamoto A, Indelman M, Shani-Adir A, Adir N, Lipkin E, Bercovici S, Geiger D, van Steensel MA, Steijlen PM, Bergman R, Bindereif A, Choder M, Shalev S, Sprecher E.
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Journal Title
Am J Hum Genet 82
Pages: 1114-21
Related Report
Peer Reviewed
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[Journal Article] Targeted deletion of themurine corneodesmosin gene delineates its essential role in skin and hair physiology.2008
Author(s)
Matsumoto M, Zhou Y, Matsuo S, Nakanishi H, Hirose K, Oura H, Arase S, Ishida-Yamamoto A, Bando Y, Izumi K, Kiyonari H, Oshima N, Nakayama R, MatsushimaA, Hirota F, Mouri Y, Kuroda N, Sano S, Chaplin DD.
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Journal Title
Proc Natl Acad Sci U S A 105
Pages: 6720-4
Related Report
Peer Reviewed
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[Journal Article] Defective lamellar granule secretion inarthrogryposis, renal dysfunction, and cholestasis syndrome caused by a mutation in VPS33B.2008
Author(s)
Hershkovitz D, Mandel H, Ishida-Yamamoto A, Chefetz I, Hino B, Luder A, Indelman M, Bergman R, Sprecher E.
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Journal Title
Arch Dermatol 144
Pages: 334-40
Related Report
Peer Reviewed
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[Journal Article] KRT14 haploinsufficiency results in increased susceptibility of keratinocytes to TNF-alpha-induced apoptosis and causes Naegeli-Franceschetti-Jadassohn syndrome.2008
Author(s)
Lugassy J, McGrath JA, Itin P, Shemer R, Verbov J, Murphy HR, Ishida-Yamamoto A, Digiovanna JJ, Bercovich D, Karin N, Vitenshtein A, Uitto J, Bergman R, Richard G, Sprecher E.
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Journal Title
J Invest Dermatol 128
Pages: 1517-24
Related Report
Peer Reviewed
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[Journal Article] Autosomal recessive ichthyosis with hypotrichosis caused by a mutation in ST14, encoding type II transmembrane serine protease matriptase.2007
Author(s)
Basel-Vanagaite L, Attia R, Ishida-Yamamoto A, Rainshtein L, Ben Amitai D, Lurie R, Pasmanik-Chor M, Indelman M, Zvulunov A, Saban S, Magal N, Sprecher E, Shohat M.
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Journal Title
Am J Hum Genet 80
Pages: 467-77
Related Report
Peer Reviewed
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[Journal Article] Kallikrein 8 is involved in skin desquamation in cooperation with other kallikreins.2007
Author(s)
Kishibe M, Bando Y, Terayama R, Namikawa K, Takahashi H, Hashimoto Y, Ishida-Yamamoto A, Jiang YP, Mitrovic B, Perez D, Iizuka H, Yoshida S.
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Journal Title
J Biol Chem 282
Pages: 5834-41
Related Report
Peer Reviewed
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