Cystatin B Polyclonal Antibody

Ab type

Primary antibody

Background

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). One type of mutation responsible for EPM1 is the expansion in the promoter region of this gene of a CCCCGCCCCGCG rep

Reactivity

Human, Rat

Clonality

Polyclonal

Host

Rabbit

Isotype

IgG

Immunogen

The antiserum was produced against synthesized peptide derived from human Stefin B. AA range:49-98

Storage Stability

-20°C for 1 year

Concentration

1 mg/ml

MolecularWeight_Da

11140

synonyms

Cystatin-B; CSTB; CST6; STFB; Cystatin-B; CPI-B; Liver thiol proteinase inhibitor; Stefin-B