Cystatin-B is a protein that in humans is encoded by the CSTB gene.[5][6]
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 cysteine 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 Unverricht–Lundborg disease, a form of progressive myoclonic epilepsy (EPM1).[6]
^ abcGRCh38: Ensembl release 89: ENSG00000160213 – Ensembl, May 2017
^ abcGRCm38: Ensembl release 89: ENSMUSG00000005054 – Ensembl, May 2017
^"Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^"Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^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. Bibcode:1996Sci...271.1731P. doi:10.1126/science.271.5256.1731. PMID 8596935. S2CID 84361089.
Cystatin-B is a protein that in humans is encoded by the CSTB gene. The cystatin superfamily encompasses proteins that contain multiple cystatin-like sequences...
Cystatin C or cystatin 3 (formerly gamma trace, post-gamma-globulin, or neuroendocrine basic polypeptide), a protein encoded by the CST3 gene, is mainly...
described below. Cystatins show similarity to fetuins, kininogens, histidine-rich glycoproteins and cystatin-related proteins. Cystatins mainly inhibit...
splice site mutation on the 3' strand was found in a gene coding for cystatinB in Progressive Myoclonus Epilepsy patients. This combination of mutations...
"Cathepsin B and cystatins: evidence for a role in cancer progression". Seminars in Cancer Biology. 1 (2): 137–52. PMID 2103490. Chan SJ, San Segundo B, McCormick...
Cystatin-A is a protein that in humans is encoded by the CSTA gene. The cystatin superfamily encompasses proteins that contain multiple cystatin-like sequences...
CRYZL1: encoing protein crystallin zeta-like 1 CSTB: encoding protein cystatin-B CXADR: encoding protein coxsackievirus and adenovirus receptor CYYR1:...
these is cystatin C, a ubiquitous protein secreted by most cells in the body (it is an inhibitor of cysteine protease).[citation needed] Cystatin C is freely...
Hereditary cystatin C amyloid angiopathy (HCCAA) is a rare, fatal type of hereditary cerebral amyloid angiopathy found almost exclusively in Iceland. A...
has autosomal recessive inheritance, and is caused by a mutation in the cystatinB (EPM1) gene on chromosome 21q22.3, which was discovered in 1996. It has...
which encodes a novel nuclear protein, maps in close proximity to the cystatinB gene within the EPM1 and APECED critical region on 21q22.3". Genomics...
Cystatin-SN is a protein that in humans is encoded by the CST1 gene. The cystatin superfamily encompasses proteins that contain multiple cystatin-like...
, Skolasky, R. L., Wojna, V., & Meléndez, L. M. (2013). Cathepsin B and cystatinB in HIV-seropositive women are associated with infection and HIV-1-associated...
Northern blot studies showed that cystatin C was produced by all nucleated human cells. The biological function of cystatin C was suggested to be inhibition...
"Role of the single cysteine residue, Cys 3, of human and bovine cystatinB (stefin B) in the inhibition of cysteine proteinases". Protein Sci. 10 (9):...
measures, which involve clearance of such substrates as EDTA, iohexol, cystatin C, 125I-iothalamate (sodium radioiothalamate), the chromium radioisotope...
Cystatin-9-like is a protein that in humans is encoded by the CST9L gene. The cystatin superfamily encompasses proteins that contain multiple cystatin-like...
diabetic nephropathy at earlier stages and identify progression risk. Cystatin C is a protein that is freely filtered in the glomeruli before it is reabsorbed...
human cystatin A (stefin A) in the binding of target proteinases. Characterization by kinetic and equilibrium methods of the interactions of cystatin A Gly-4...
(September 2007). "Affilin-novel binding molecules based on human gamma-B-crystallin, an all beta-sheet protein". J. Mol. Biol. 372 (1): 172–85. doi:10...
involving other amyloid peptides: the "Icelandic type" is associated with cystatin C amyloid (ACys). the "British type" and "Danish type" are associated with...
that evolved from the protein cystatin by gene duplication and exchange of gene segments. Fetuins thus belong to the cystatin superfamily of proteins. Fetuin...
diagnosed by checking the levels of several biomarkers such as clusterin and cystatin C. While the duration of ischemia was used as a biomarker, it was found...
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