Molecular Analysis of p94 and Its Application to Diagnosis of Limb Girdle Muscular Dystrophy Type 2A

  • Hiroyuki Sorimachi
  • Yasuko Ono
  • Koichi Suzuki
Part of the Methods in Molecular Biology™ book series (MIMB, volume 144)


p94 (also called calpain 3, nCL-1, or CAPN3) is a calpain large subunit homologue, which is predominantly expressed in skeletal muscle (1, 2, 3, 4). The mRNA level of p94 in skeletal muscle is at least 10 times higher than that for the conventional calpain subunits (1). However, the p94 protein is not easily detectable, since p94 undergoes very rapid and extensive autolysis immediately after translation, and its half-life in vitro is less than 10 minutes (5). Thus, analysis of p94 at the protein level is very difficult.


Muscular Dystrophy COS7 Cell Limb Girdle Muscular Dystrophy Autolytic Activity Limb Girdle Muscular Dystrophy Type 
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Copyright information

© Humana Press Inc. 2000

Authors and Affiliations

  • Hiroyuki Sorimachi
    • 1
  • Yasuko Ono
    • 1
  • Koichi Suzuki
    • 1
  1. 1.Laboratory of Molecular Structure and Function, Institute of Molecular and Cellular BiosciencesUniversity of TokyoTokyoJapan

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