Calpain and pathology in view of structure-function relationships

• SORIMACHI Hiroyuki

  Laboratory of Biological Function, Dept. of Applied Biological Chemistry, Graduate School of Agricultural and Life Sciences, The University of Tokyo

• KAWABATA Yukiko

  Laboratory of Biological Function, Dept. of Applied Biological Chemistry, Graduate School of Agricultural and Life Sciences, The University of Tokyo

Bibliographic Information

Other Title

• カルパインと病態―構造活性相関からの考察―
• カルパイン ト ビョウタイ コウゾウ カッセイ ソウカン カラ ノ コウサツ
• −構造活性相関からの考察−

Abstract

Calpain, a Ca²⁺-requiring cytoplasmic cysteine protease, plays indispensable roles in various cellular functions such as signal transduction, cell growth and differentiation, apoptosis, necrosis, and so on. Although most of the detailed physiological functions of calpains have not yet been elucidated, the importance of calpain is obvious from the increasing numbers of papers describing relationships between human disease states (such as Alzheimer's disease, cataract, and muscular dystrophies) and malfunction of calpain. One of the recent remarkable topics of calpain is that a single nucleotide polymorphism of CAPN10, the gene for calpain 10, is related to type 2 diabetes. However, physiological functions of calpain 10 and its relation to diabetes are still unclear. Among 14 human calpain genes, mutations in CAPN3, the gene for p94/calpain 3a and Lp82/calpain 3b, are the only example that genetically connects the calpain gene and human disease, in this case, limb-girdle muscular dystrophy type 2A (LGMD2A). p94 has unique characteristics such as apparent Ca²⁺-independent activation and very rapid autolytic activity, which are dependent on p94-specific regions, NS, IS1, and IS2. Based on the 3D structures of µ- and m-calpain, molecular functions of p94 in relation to LGMD2A are discussed, with the hope of providing us with some clues to understand calpain functions and its relationships to human diseases.<br>

Journal

• Folia Pharmacologica Japonica

  Folia Pharmacologica Japonica 122 (1), 21-29, 2003

  The Japanese Pharmacological Society

Keywords

• calpain
• muscular dystrophy
• 3D structure
• proteolysis
• autolysis

Details

• CRID: 1390001204273588992
• NII Article ID: 130000085994
• NII Book ID: AN00198335
• DOI: 10.1254/fpj.122.21
• COI: 1:STN:280:DC%2BD3szhsFShug%3D%3D
• ISSN: 13478397
• ISSN: 00155691
• NDL BIB ID: 6630022
• PubMed: 12843569
• Web Site: https://ndlsearch.ndl.go.jp/books/R000000004-I6630022
• Web Site: https://search.jamas.or.jp/link/ui/2004066696
• Text Lang: ja
• Data Source:
  • JaLC
  • NDL
  • Crossref
  • PubMed
  • CiNii Articles
  • KAKEN
• Abstract License Flag: Disallowed