胱抑素B
位於21號人類染色體的基因
胱抑素B(英文:Cystatin-B)是一种在人体中由CSTB基因编码的蛋白质。[6][7]
![](http://images.weserv.nl/?url=//upload.wikimedia.org/wikipedia/commons/thumb/a/a0/1stf.jpg/300px-1stf.jpg)
胱抑素超家族包括含有多个胱抑素样序列的蛋白质。一些成员是活性半胱氨酸蛋白酶抑制剂,而其他成员已经失去或可能从未获得这种抑制活性。超家族中有3个抑制家族,包括1型胱抑素(stefin)、2型胱抑素和激肽原。该基因编码一种作为细胞内半胱氨酸蛋白酶抑制剂而起作用的stefin。胱抑素B能够形成由非共价作用力稳定的二聚体,抑制木瓜蛋白酶和组织蛋白酶L1、H和B。胱抑素B被认为在防止蛋白酶从溶酶体泄漏方面发挥作用。有证据表明,该基因突变是导致进行性肌阵挛性癫痫患者出现主要缺陷的原因。[7]
交互作用
参考文献
- ^ 與血清胱抑素B相關的疾病;在維基數據上查看/編輯參考.
- ^ 2.0 2.1 2.2 GRCh38: Ensembl release 89: ENSG00000160213 - Ensembl, May 2017
- ^ 3.0 3.1 3.2 GRCm38: 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. Mutations in the gene encoding cystatin B in progressive myoclonus epilepsy (EPM1). Science. Apr 1996, 271 (5256): 1731–4. Bibcode:1996Sci...271.1731P. PMID 8596935. S2CID 84361089. doi:10.1126/science.271.5256.1731.
- ^ 7.0 7.1 Entrez Gene: CSTB cystatin B (stefin B).
- ^ Pavlova, Alona; Björk Ingemar. 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). Sep 2003, 42 (38): 11326–33. ISSN 0006-2960. PMID 14503883. doi:10.1021/bi030119v.
- ^ Pol, E; Björk I. 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). Sep 2001, 10 (9): 1729–38. ISSN 0961-8368. PMC 2253190 . PMID 11514663. doi:10.1110/ps.11901.
拓展阅读
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