Stefin B (CSTB) Mouse Monoclonal Antibody (Biotin conjugated) [Clone ID: OTI1E8]

Specifications

Product Data
Clone Name	OTI1E8
Applications	IHC, WB
Recommend Dilution	WB 1:500, IHC 1:500
Reactivity	Human
Host	Mouse
Clonality	Monoclonal
Immunogen	Human recombinant protein fragment corresponding to amino acids 1-98 of human CSTB (NP_000091) produced in E.coli.
Formulation	PBS (pH 7.3) containing 1% BSA, 50% glycerol and 0.02% sodium azide.
Concentration	0.5 mg/ml
Purification	Purified from mouse ascites fluids or tissue culture supernatant by affinity chromatography (protein A/G)
Conjugation	Biotin
Storage Condition	Store at -20°C as received.
Predicted Protein Size	11 kDa
Gene Name	cystatin B
Database Link	NP_000091 Entrez Gene 1476 Human UniProt ID P04080
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 repeat from 2-3 copies to 30-78 copies. [provided by RefSeq, Jul 2016]
Synonyms	CPI-B; CST6; EPM1; EPM1A; PME; STFB; ULD
Reference Data

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