Frontiers of model animals for human diseases: Expanding members and roles of the calpain superfamily and their genetically modified animals

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  • SORIMACHI Hiroyuki
    Calpain Project, The Tokyo Metropolitan Institute of Medical Science (Rinshoken)
  • HATA Shoji
    Calpain Project, The Tokyo Metropolitan Institute of Medical Science (Rinshoken)
  • ONO Yasuko
    Calpain Project, The Tokyo Metropolitan Institute of Medical Science (Rinshoken)

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タイトル別名
  • Expanding Members and Roles of the Calpain Superfamily and Their Genetically Modified Animals

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抄録

Calpains are intracellular Ca2+-dependent cysteine proteases (Clan CA, family C02, EC 3.4.22.17) found in almost all eukaryotes and some bacteria. Calpains display limited proteolytic activity at neutral pH, proteolysing substrates to transform and modulate their structures and activities, and are therefore called “modulator proteases”. The human genome has 15 genes that encode a calpain-like protease domain, generating diverse calpain homologues that possess combinations of several functional domains such as Ca2+-binding domains and Zn-finger domains. The importance of the physiological roles of calpains is reflected in the fact that particular defects in calpain functionality cause a variety of deficiencies in many different organisms, including lethality, muscular dystrophies, lissencephaly, and tumorigenesis. In this review, the unique characteristics of this distinctive protease superfamily are introduced in terms of genetically modified animals, some of which are animal models of calpain deficiency diseases.<br>

収録刊行物

  • Experimental Animals

    Experimental Animals 59 (5), 549-566, 2010

    公益社団法人 日本実験動物学会

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