Cystatin B rabbit pAb

SKU: ES8767

Size: 100μL

Source:Rabbit

Applications:IHC;IF;ELISA

Reactivity:Human;Rat;Mouse;

Dilution:IHC-p 1:50-200, ELISA 1:10000-20000

Immunogen:Synthetic peptide from human protein at AA range: 20-60

Storage_stability:-20°C/1 year

Clonality:Polyclonal

Isotype:IgG

Concentration:1 mg/ml

Observed_band(KD):

Human_gene_id:1476

Human_swiss_prot_no:P04080

Subcellular_location:Cytoplasm . Nucleus .

Other_name:Cystatin-B (CPI-B;Liver thiol proteinase inhibitor;Stefin-B)

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