Cystatin B


Cystatin B
CSTB redirects here. It can also refer to the Trade Union Confederation of Bolivian Workers.
Cystatin B (stefin B)

PDB rendering based on 1stf.
Identifiers
Symbols CSTB; CST6; EPM1; PME; STFB
External IDs OMIM601145 MGI109514 HomoloGene79 GeneCards: CSTB Gene
RNA expression pattern
PBB GE CSTB 201201 at tn.png
More reference expression data
Orthologs
Species Human Mouse
Entrez 1476 13014
Ensembl ENSG00000160213 ENSMUSG00000005054
UniProt P04080 Q62426
RefSeq (mRNA) NM_000100 NM_007793.3
RefSeq (protein) NP_000091 NP_031819.1
Location (UCSC) Chr 21:
45.19 – 45.2 Mb
Chr 10:
77.89 – 77.89 Mb
PubMed search [1] [2]

Cystatin-B is a protein that in humans is encoded by the CSTB gene.[1][2]

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).[2]

Contents

Interactions

Cystatin B has been shown to interact with Cathepsin B.[3][4]

References

  1. ^ Pennacchio LA, Lehesjoki AE, Stone NE, Willour VL, Virtaneva K, Miao J, D'Amato E, Ramirez L, Faham M, Koskiniemi M, Warrington JA, Norio R, de la Chapelle A, Cox DR, Myers RM (Apr 1996). "Mutations in the gene encoding cystatin B in progressive myoclonus epilepsy (EPM1)". Science 271 (5256): 1731–4. doi:10.1126/science.271.5256.1731. PMID 8596935. 
  2. ^ a b "Entrez Gene: CSTB cystatin B (stefin B)". http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=1476. 
  3. ^ Pavlova, Alona; Björk Ingemar (Sep. 2003). "Grafting of features of cystatins C or B into the N-terminal region or second binding loop of cystatin A (stefin A) substantially enhances inhibition of cysteine proteinases". Biochemistry (United States) 42 (38): 11326–33. doi:10.1021/bi030119v. ISSN 0006-2960. PMID 14503883. 
  4. ^ Pol, E; Björk I (Sep. 2001). "Role of the single cysteine residue, Cys 3, of human and bovine cystatin B (stefin B) in the inhibition of cysteine proteinases". Protein Sci. (United States) 10 (9): 1729–38. doi:10.1110/ps.11901. ISSN 0961-8368. PMC 2253190. PMID 11514663. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2253190. 

Further reading

External Links


Wikimedia Foundation. 2010.

Look at other dictionaries:

  • Cystatin C — PDB rendering based on 1r4c …   Wikipedia

  • Cystatin A — (stefin A) PDB rendering based on 1cyu …   Wikipedia

  • Cystatin — C nach PDB  …   Deutsch Wikipedia

  • Cystatin C — nach PDB  …   Deutsch Wikipedia

  • Cystatin — Proteinase inhibitor I25, cystatin Crystal structure of an immunomodulatory salivary cystatin from the soft tick Ornithodoros moubata.[1] …   Wikipedia

  • cystatin — noun Any of a group of polypeptides that are cysteine protease inhibitors …   Wiktionary

  • cystatin C — cys·tat·in C (sis tatґin) an inhibitor of cysteine endopeptidase, produced by most cells in the body and excreted into the bloodstream; it is freely filtered by the renal glomeruli and its rate of clearance can be used to calculate the glomerular …   Medical dictionary

  • CST2 — Cystatin SA, also known as CST2, is a human gene.cite web | title = Entrez Gene: CST2 cystatin SA| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene Cmd=ShowDetailView TermToSearch=1470| accessdate = ] PBB Summary section title = summary… …   Wikipedia

  • CST4 — Cystatin S, also known as CST4, is a human gene.cite web | title = Entrez Gene: CST4 cystatin S| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene Cmd=ShowDetailView TermToSearch=1472| accessdate = ] PBB Summary section title = summary text …   Wikipedia

  • CST1 — Cystatin SN, also known as CST1, is a human gene.cite web | title = Entrez Gene: CST1 cystatin SN| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene Cmd=ShowDetailView TermToSearch=1469| accessdate = ] PBB Summary section title = summary… …   Wikipedia


Share the article and excerpts

Direct link
Do a right-click on the link above
and select “Copy Link”

We are using cookies for the best presentation of our site. Continuing to use this site, you agree with this.