Human CSTB Recombinant Protein (RPPB3300)
- SKU:
- RPPB3300
- Product type:
- Recombinant Protein
- Size:
- 20ug
- Species:
- Human
- Target:
- CSTB
- Synonyms:
- Cystatin-B
- Stefin-B
- Liver thiol proteinase inhibitor
- CPI-B
- Source:
- Escherichia Coli
- Uniprot:
- P04080
Description
| Product Name: | Human CSTB Recombinant Protein |
| Product Code: | RPPB3300 |
| Size: | 20µg |
| Species: | Human |
| Target: | CSTB |
| Synonyms: | Cystatin-B, Stefin-B, Liver thiol proteinase inhibitor, CPI-B, CSTB, CST6, EPM1, PME, STFB. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile Filtered colorless solution. |
| Formulation: | The protein solution contains 20mM Tris-HCl pH-8 & 50mM NaCl. |
| Stability: | Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°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. |
| Purity: | Greater than 95.0% as determined by SDS-PAGE. |
| Amino Acid Sequence: | MGSSHHHHHH SSGLVPRGSH MMCGAPSATQ PATAETQHIA DQVRSQLEEK ENKKFPVFKA VSFKSQVVAG TNYFIKVHVG DEDFVHLRVF QSLPHENKPL TLSNYQTNKA KHDELTYF |
Type 1 cystatins are also called stefins which function as intracellular thiol protease inhibitors. Cystatin-B protein is able to form a dimer stabilized by noncovalent forces, inhibiting papain and cathepsins l, h and b. CSTB protein protects proteases leakage from lysosomes. Mutations in Stefin-B gene cause primary defects in patients with progressive myoclonic epilepsy (EPM1), a degenerative disease of the central nervous system. CSTB is overexpressed & elevated in the serum of HCC patients. Cystatin-B in vivo has a polymeric structure which is sensitive to the redox environment. Cystatin-B inhibits bone resorption by down-regulating intracellular cathepsin K activity despite increased osteoclast survival. Protein and mRNA levels of stefin B are significantly lower in atypical benign meningiomas. Stefins-A & Stefin-B which belong to the type-1 Cystatins, are up-regulated in lung tumours and thus able to counteract harmful tumour-associated proteolytic activity. Human stefin-A & Stefin-B form amyloid fibrils. Copper binding by stefin-B reduces amyloid fibril formation.A number of alternatively spliced CSTB isoforms were recognized in patients with progressive myoclonus epilepsy. Decreased CSTB activity in EPM1 pathogenesis is controled by cathepsins through increased activity of cathepsin-S & cathepsin-L.
CSTB Human Recombinant fused to His-Tag at N-Terminus produced in E.Coli is a single, non-glycosylated polypeptide chain containing 118 amino acids and having a molecular mass of 13 kDa.
| UniProt Protein Function: | 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. |
| UniProt Protein Details: | 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 |
| NCBI 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). [provided by RefSeq, Jul 2008] |
| UniProt Code: | P04080 |
| NCBI GenInfo Identifier: | 1706278 |
| NCBI Gene ID: | 1476 |
| NCBI Accession: | P04080.2 |
| UniProt Related Accession: | P04080 |
| Molecular Weight: | 11,140 Da |
| NCBI Full Name: | Cystatin-B |
| NCBI 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 |
| UniProt Synonym Protein Names: | CPI-B; Liver thiol proteinase inhibitor; Stefin-B |
| Protein Family: | Cystatin |
| UniProt Gene Name: | CSTB |
| UniProt Entry Name: | CYTB_HUMAN |
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