[HTML][HTML] Insertion sequence 1 of muscle-specific calpain, p94, acts as an internal propeptide
BG Diaz, T Moldoveanu, MJ Kuiper… - Journal of Biological …, 2004 - ASBMB
The physiological role of the skeletal muscle-specific calpain 3, p94, is presently unknown,
but defects in its gene cause limb girdle muscular dystrophy type 2A. This calcium-
dependent cysteine protease resembles the large subunit of m-calpain but with three unique
additional sequences: an N-terminal region (NS), and two insertions (IS1 and IS2). The latter
two insertions have been linked to the chronic instability of the whole enzyme both in vivo
and in vitro. We have shown previously that the core of p94 comprising NS, domains I and II …
but defects in its gene cause limb girdle muscular dystrophy type 2A. This calcium-
dependent cysteine protease resembles the large subunit of m-calpain but with three unique
additional sequences: an N-terminal region (NS), and two insertions (IS1 and IS2). The latter
two insertions have been linked to the chronic instability of the whole enzyme both in vivo
and in vitro. We have shown previously that the core of p94 comprising NS, domains I and II …