CAPN3
Reactivité: Humain
WB, ELISA, IHC
Hôte: Lapin
Polyclonal
unconjugated
Indications d'application
ELISA, Western blotting: 1µg/ml for 2hrs.
Restrictions
For Research Use only
Format
Liquid
Buffer
This antibody is stored in PBS, 50% glycerol
Agent conservateur
Sodium azide
Précaution d'utilisation
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Stock
-20 °C
Antigène
Calpain 3 (CAPN3)
Autre désignation
CAPN3
Sujet
CAPN3(Calpain-3) is a calcium-regulated non-lysosomal thiol-protease. It has a broad endopeptidase specificity. CAPN3 is activated by micromolar concentrations of calcium and inhibited by calpastatin. It interacts with TTN/titin. Isoform I is skeletal muscle specific. Defects in CAPN3 are the cause of limb-girdle muscular dystrophy type 2A (LGMD2A). LGMD2A is both autosomal dominantly and recessively transmitted. It is characterized by progressive symmetrical atrophy and weakness of the proximal limb muscles and elevated serum creatine kinase. CAPN3 belongs to the peptidase C2 family and contains 1 calpain catalytic domain and 4 EF-hand domains.