ヒト疾患モデル動物の最前線 拡大し続けるカルパイン・スーパーファミリー分子--その機能と遺伝子改変動物 Expanding Members and Roles of the Calpain Superfamily and Their Genetically Modified Animals

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Author(s)

Abstract

Calpains are intracellular Ca<sup>2+</sup>-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 Ca<sup>2+</sup>-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>

Journal

  • Experimental Animals

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

    Japanese Association for Laboratory Animal Science

Cited by:  2

Codes

  • NII Article ID (NAID)
    130000419250
  • NII NACSIS-CAT ID (NCID)
    AA11032321
  • Text Lang
    ENG
  • Article Type
    Journal Article
  • ISSN
    1341-1357
  • NDL Article ID
    10838115
  • NDL Source Classification
    ZS7(科学技術--医学)
  • NDL Call No.
    Z54-H752
  • Data Source
    CJPref  NDL  J-STAGE 
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