Cystatin-B Active Protein | CSTB active protein

Recombinant Human Cystatin-B, BioActive

Cat. Num. AAA141619

• Gene Names: CSTB; PME; ULD; CST6; EPM1; STFB; EPM1A
• Purity: Greater than 95.0% as determined by SDS-PAGE.
• Synonyms: Cystatin-B; Recombinant Human Cystatin-B; BioActive; CSTB Active; CSTB Human Active; BioActive Human Recombinant; Stefin-B; Liver thiol proteinase inhibitor; CPI-B; CSTB; CST6; EPM1; PME; STFB; CSTB active protein

• Host: E Coli
• Purity/Purification: Greater than 95.0% as determined by SDS-PAGE.
• Form/Format: Sterile filtered colorless solution.; The CSTB solution (1mg/1ml) contains 50mM NaCl and 20mM Tris-HCl buffer (pH8.0).
• Sequence: MGSSHHHHHH SSGLVPRGSH MMCGAPSATQ PATAETQHIA DQVRSQLEEK ENKKFPVFKA VSFKSQVVAG TNYFIKVHVG DEDFVHLRVF QSLPHENKPL TLSNYQTNKA KHDELTYF

• Biological Activity: The IC50 value is <3.0nM. The inhibitory function of Cystatin B on protease activity of papain was measured by a fluorometric assay using Z-FR-AMC at pH7.5 at 25 degree C.
• Preparation and Storage: Store at 4 degree C if entire vial will be used within 2-4 weeks.; Store, frozen at -20 degree C for longer periods of time.; For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA). Avoid multiple freeze-thaw cycles.

Related Product Information for CSTB active protein

Cystatin B or CSTB, is an protein (anti-protease) that is suspected to be involved in myoclonus epilepsy. As part of the encompasses proteins family, it has many cystatin-like sequences. Part of the cystatin superfamily are cysteine protease inhibitors; part don't have the ability. CSTB can create a dimer that is stable because of noncovalent bonds and has a crucial part in shielding from lysosomal proteases leaking. Cystatin B can be found in the lysosomes, nucleus & the cytoplasm in cells.
CSTB Human Recombinant produced in E Coli is a single, non-glycosylated polypeptide chain containing 118 amino acids (1-98 a.a.) and having a molecular mass of 13kDa.
CSTB is fused to a 20 amino acid His tag at N-Terminus and purified by proprietary chromatographic techniques.

Product Categories/Family for CSTB active protein

RECOMBINANT & NATURAL PROTEINS

NCBI and Uniprot Product Information

• NCBI GI #: 4503117
• NCBI GeneID: 1476
• NCBI Accession #: NP_000091.1
• NCBI GenBank Nucleotide #: NM_000100.3
• UniProt Accession #: P04080
• Molecular Weight: 11,140 Da
• NCBI Official Full Name: cystatin-B
• NCBI Official Synonym Full Names: cystatin B (stefin B)
• NCBI Official Symbol: CSTB
• NCBI Official Synonym Symbols: PME; ULD; CST6; EPM1; STFB; EPM1A
• NCBI Protein Information: cystatin-B; CPI-B; liver thiol proteinase inhibitor
• UniProt Protein Name: Cystatin-B
• Protein Family: Cystatin
• UniProt Gene Name: CSTB
• UniProt Synonym Gene Names: CST6; STFB
• UniProt Entry Name: CYTB_HUMAN

NCBI Description

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]

Uniprot Description

CSTB: This is an intracellular thiol proteinase inhibitor. Tightly binding reversible inhibitor of cathepsins L, H and B. Defects in CSTB are the cause of progressive myoclonic epilepsy type 1 (EPM1). EPM1 is an autosomal recessive disorder characterized by severe, stimulus-sensitive myoclonus and tonic-clonic seizures. The onset, occurring between 6 and 13 years of age, is characterized by convulsions. Myoclonus begins 1 to 5 years later. The twitchings occur predominantly in the proximal muscles of the extremities and are bilaterally symmetrical, although asynchronous. At first small, they become late in the clinical course so violent that the victim is thrown to the floor. Mental deterioration and eventually dementia develop. Belongs to the cystatin family.

Protein type: Inhibitor

Chromosomal Location of Human Ortholog: 21q22.3

Cellular Component: extracellular space; cytoplasm; nucleolus

Molecular Function: protease binding; endopeptidase inhibitor activity; cysteine protease inhibitor activity

Biological Process: negative regulation of proteolysis; regulation of apoptosis; adult locomotory behavior; negative regulation of peptidase activity

Disease: Myoclonic Epilepsy Of Unverricht And Lundborg