Analysis of cellular molecules associated with keratinocyte differentiation

与角质形成细胞分化相关的细胞分子分析

基本信息

  • 批准号:
    14570794
  • 负责人:
  • 金额:
    $ 2.24万
  • 依托单位:
  • 依托单位国家:
    日本
  • 项目类别:
    Grant-in-Aid for Scientific Research (C)
  • 财政年份:
    2002
  • 资助国家:
    日本
  • 起止时间:
    2002 至 2005
  • 项目状态:
    已结题

项目摘要

We studied peptidylarginine deiminases (PADs) expression in human epidermis (Cell Mol Life Sci 2005). We found that PAD1,2 and 3 are expressed. PAD1 and PAD3 are co-localized with filaggrin. PAD was present up to the supper corneocytes where it deiminates keratin K1.We have shown that LEKI is localized in lamellar granules, separated from KLK5 and KLK7, and is secreted in the extracellular spaces of the superficial stratum granulosum in the epidermis (J Invest Dermatol 2005).We also disclosed that in Netherton syndrome where LEKTI is absent, an abnormal split was seen in the superficial stratum granulosum, suggesting that LEKTI is preventing premature loss of stratum corneum cohesion.We generated SPINK5-deficient mice as a model for Netherton syndrome. Using this model, we showed that desmosome cleavage due to desmoglein 1 degradation is the primary pathogenic event in this disease. (Nature Genetics 2005).We describe a novel neurocutaneous syndrome characterized by cerebral dysgenesis, neuropathy, ichthyosis, and keratoderma (CEDNIK syndrome) caused by a mutation in SNAP29, coding for a SNARE protein involved in intracellular trafficking (Am J Hum Genet 2005). In the ichthyotic patient skin, secretion of lamellar granule proteins was inhibited.We have identified novel members of lamellar granule proteins, namely A2ML (alpha-2 macroglobulin-like) and Dermokin (J Biol Chem, 2006,J Invest Dermatol 2006).
我们研究了人表皮中肽精氨酸脱亚胺酶(PADs)的表达(Cell Mol Life Sci 2005)。我们发现PAD1,2和3表达。PAD1和PAD3与聚丝蛋白共定位。PAD存在于晚餐角质细胞,在那里它消除角蛋白K1。我们已经证明LEKI定位于板层颗粒中,与KLK5和KLK7分离,并在表皮浅层颗粒的细胞外间隙分泌(J Invest Dermatol 2005)。我们还发现,在缺乏LEKTI的内德顿综合征中,在浅层颗粒中可以看到异常分裂,这表明LEKTI可以防止角质层粘连的过早丧失。我们制造了spink5缺陷小鼠作为内瑟顿综合征的模型。通过该模型,我们发现由桥粒蛋白1降解引起的桥粒分裂是该疾病的主要致病事件。(Nature Genetics, 2005)。我们描述了一种新的神经皮肤综合征,其特征是大脑发育不良、神经病变、鱼鳞病和角膜炎(CEDNIK综合征),由SNAP29突变引起,编码参与细胞内运输的SNARE蛋白(Am J Hum Genet 2005)。在鱼鳞病患者皮肤中,板层颗粒蛋白的分泌受到抑制。我们已经确定了板层颗粒蛋白的新成员,即A2ML (α -2巨球蛋白样蛋白)和Dermokin (J Biol Chem, 2006,J Invest Dermatol 2006)。

项目成果

期刊论文数量(77)
专著数量(0)
科研奖励数量(0)
会议论文数量(0)
专利数量(0)
Periplakin gene targeting reveals a constituent of the cornified cell envelope dispensable for normal mouse development
  • DOI:
    10.1128/mcb.24.14.6410-6418.2004
  • 发表时间:
    2004-07-01
  • 期刊:
  • 影响因子:
    5.3
  • 作者:
    Aho, S;Li, KH;Klement, JF
  • 通讯作者:
    Klement, JF
Akemi Ishida-Yamamoto, et al.: "Lessons from disorders of epidermal differentiatin-associated keratins"Histology and Histopathology. 17. 331-338 (2002)
Akemi Ishida-Yamamoto 等人:“表皮分化相关角蛋白疾病的教训”组织学和组织病理学。
  • DOI:
  • 发表时间:
  • 期刊:
  • 影响因子:
    0
  • 作者:
  • 通讯作者:
Immunolocalization of target autoantigens in IgA pemphigus
  • DOI:
    10.1111/j.1365-2230.2004.01436.x
  • 发表时间:
    2004-01-01
  • 期刊:
  • 影响因子:
    4.1
  • 作者:
    Ishii, N;Ishida-Yamamoto, A;Hashimoto, T
  • 通讯作者:
    Hashimoto, T
Akemi Ishida-Yamamoto: "Loricrin keratoderma. A novel disease entity characterized by nuclear accumulation of mutant loricrin"Journal of Dermatological Science. 31. 3-8 (2003)
Akemi Ishida-Yamamoto:“Loricrin keratoderma。一种以突变型 Loricrin 核积累为特征的新型疾病实体”皮肤病学杂志。
  • DOI:
  • 发表时间:
  • 期刊:
  • 影响因子:
    0
  • 作者:
  • 通讯作者:
Prediction of a coding sequence for a novel type II keratin from N-terminal sequences of mouse epidermal proteins site-specifically deiminated in embryonic development.
从胚胎发育过程中位点特异性脱亚胺化的小鼠表皮蛋白的 N 端序列预测新型 II 型角蛋白的编码序列。
  • DOI:
  • 发表时间:
    2005
  • 期刊:
  • 影响因子:
    0
  • 作者:
    Senshu T;Ishida-Yamamoto A;Takahashi H;Iizuka H.
  • 通讯作者:
    Iizuka H.
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YAMAMOTO Akemi其他文献

YAMAMOTO Akemi的其他文献

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{{ truncateString('YAMAMOTO Akemi', 18)}}的其他基金

Pathological mechanisms of keratinization abnormalities
角化异常的病理机制
  • 批准号:
    19591294
  • 财政年份:
    2007
  • 资助金额:
    $ 2.24万
  • 项目类别:
    Grant-in-Aid for Scientific Research (C)
Cell biological analyses of cornified cell envelope formation
角质化细胞包膜形成的细胞生物学分析
  • 批准号:
    10470184
  • 财政年份:
    1998
  • 资助金额:
    $ 2.24万
  • 项目类别:
    Grant-in-Aid for Scientific Research (B).
Expression and Abnormalities of Keratinocyte Terminal Differentiation-Associated Proteins
角质形成细胞终末分化相关蛋白的表达及异常
  • 批准号:
    08670940
  • 财政年份:
    1996
  • 资助金额:
    $ 2.24万
  • 项目类别:
    Grant-in-Aid for Scientific Research (C)

相似国自然基金

三角帆蚌丝氨酸蛋白酶(serine protease)基因的克隆、表达调控与功能研究
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    31040083
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