Stefin B (CSTB) (NM_000100) Human Mass Spec Standard

Specifications

Product Data
Description	CSTB MS Standard C13 and N15-labeled recombinant protein (NP_000091)
Species	Human
Expression Host	HEK293
Expression cDNA Clone or AA Sequence	RC203872
Predicted MW	11.1 kDa
Protein Sequence	Sequence Link (showhide) >RC203872 protein sequence Red=Cloning site Green=Tags(s) MMCGAPSATQPATAETQHIADQVRSQLEEKENKKFPVFKAVSFKSQVVAGTNYFIKVHVGDEDFVHLRVF QSLPHENKPLTLSNYQTNKAKHDELTYF TRTRPLEQKLISEEDLAANDILDYKDDDDKV
Tag	C-Myc/DDK
Purity	> 80% as determined by SDS-PAGE and Coomassie blue staining
Concentration	>0.05 µg/µL as determined by microplate BCA method
Labeling Method	Labeled with [U- 13C6, 15N4]-L-Arginine and [U- 13C6, 15N2]-L-Lysine
Buffer	25 mM Tris-HCl, 100 mM glycine, pH 7.3
Reference Data
RefSeq	NP_000091
RefSeq Size	940
RefSeq ORF	294
Synonyms	CPI-B; CST6; EPM1; EPM1A; PME; STFB; ULD
Locus ID	1476
Cytogenetics	21q22.3
Summary	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]

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