免疫原
Synthesized peptide derived from part region of human protein
特异性
CAN3 Polyclonal Antibody detects endogenous levels of protein.
来源
Polyclonal, Rabbit,IgG
组成（Formulation）
Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.
稀释比例
WB 1:500-2000 ELISA 1:5000-20000
纯化工艺（Immunogen）
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
背景（Background）
Calpain, a heterodimer consisting of a large and a small subunit, is a major intracellular protease, although its function has not been well established. This gene encodes a muscle-specific member of the calpain large subunit family that specifically binds to titin. Mutations in this gene are associated with limb-girdle muscular dystrophies type 2A. Alternate promoters and alternative splicing result in multiple transcript variants encoding different isoforms and some variants are ubiquitously expressed. [provided by RefSeq, Jul 2008],
功能
catalytic activity:Broad endopeptidase activity.,disease:Defects in CAPN3 are the cause of limb-girdle muscular dystrophy type 2A (LGMD2A) [MIM:253600]. LGMD2A is an autosomal recessive degenerative myopathy characterized by progressive symmetrical atrophy and weakness of the proximal limb muscles and elevated serum creatine kinase. The symptoms usually begin during the first two decades of life, and the disease gradually worsens, often resulting in loss of walking ability 10 or 20 years after onset.,enzyme regulation:Activated by micromolar concentrations of calcium and inhibited by calpastatin.,function:Calcium-regulated non-lysosomal thiol-protease.,online information:Calpain-3 mutations in LGMD2A,similarity:Belongs to the peptidase C2 family.,similarity:Contains 1 calpain catalytic domain.,similarity:Contains 4 EF-hand domains.,subunit:Interacts with TTN/titin.,tissue specificity:Iso
基因名称（Gene Name）
CAPN3 CANP3 CANPL3 NCL1
蛋白名称
Calpain-3 (EC 3.4.22.54) (Calcium-activated neutral proteinase 3) (CANP 3) (Calpain L3) (Calpain p94) (Muscle-specific calcium-activated neutral protease 3) (New calpain 1) (nCL-1)
细胞定位
Cytoplasm. Nucleus, nucleolus .
组织表达
Isoform I is skeletal muscle specific.
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