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Rabbit Anti-Cystatin B antibody
The cystatin superfamily encompasses proteins that contain multiple cystatin-like sequences. Some of the members are active cysteine protease inhibitors, while others have lost or perhaps never acquired this inhibitory activity. There are three inhibitory families in the superfamily, including the type 1 cystatins (stefins), type 2 cystatins and kininogens. This gene encodes a stefin that functions as an intracellular thiol protease inhibitor. The protein is able to form a dimer stabilized by noncovalent forces, inhibiting papain and cathepsins l, h and b. The protein is thought to play a role in protecting against the proteases leaking from lysosomes. Evidence indicates that mutations in this gene are responsible for the primary defects in patients with progressive myoclonic epilepsy (EPM1). [provided by RefSeq, Jul 2008]
Function:
Shows moderate inhibition of cathepsin B but is not active against cathepsin C.
Subcellular Location:
Cytoplasm. Nucleus.Secreted.
Tissue Specificity:
Restricted to the stratum granulosum of normal skin, the stratum granulosum/spinosum of psoriatic skin, and the secretory coils of eccrine sweat glands. Low expression levels are found in the nasal cavity.
Post-translational modifications:
Substrate for transglutaminases. Acts as an acyl acceptor but not as an acyl donor.
Similarity:
Belongs to the cystatin family.
SWISS:
P04080
Gene ID:
1476
Database links:
Entrez Gene: 1476 Human
Entrez Gene: 13014 Mouse
Entrez Gene: 25308 Rat
Omim: 601145 Human
SwissProt: P04080 Human
SwissProt: Q76LA1 Human
SwissProt: Q62426 Mouse
SwissProt: P01041 Rat
Unigene: 695 Human
Unigene: 6095 Mouse
Unigene: 1233 Rat
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