产品名称
CAN3 Rabbit Polyclonal Antibody
基因名称
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)
存储缓冲液
Liquid in PBS containing 50% glycerol, and 0.02% New type preservative N.
Human Swissprot No.
P20807
Human Swissprot Link
https://www.uniprot.org/uniprotkb/P20807/entry
Mouse Swissprot No.
Q64691
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q64691
Rat Swissprot Link
http://www.uniprot.org/uniprot/O54941P16259
免疫原
Synthesized peptide derived from part region of human protein
特异性
CAN3 Polyclonal Antibody detects endogenous levels of protein.
稀释度
WB 1:500-2000 ELISA 1:5000-20000
宿主
Polyclonal, Rabbit,IgG
背景介绍
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],
组织表达
Isoform I is skeletal muscle specific.
细胞定位
Cytoplasm. Nucleus, nucleolus .
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:Isoform I is skeletal muscle specific.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
