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Function
Syntrophins (Syn) are a family of membrane-associated adaptor proteins which are important components of many signaling events: regulating the postsynaptic signal transduction, sarcolemmal localization of brain NOS and G-protein mediated signaling [1].
- alpha-1-syntrophin is a cytoskeletal protein interacting with cardiac sodium channel[2].
- beta-1-syntrophin is a scaffold protein that provides a platform for the formation of signal transduction complexes[3].
Disease
Mutations in Syn which interacts with sodium channel cause the inherited disorder long-QT syndrome resulting in sudden cardiac death[4].
Structural highlights
Syn is characterized by a plekstrin homology domain(PH) which is split by a PDZ domain and a C-terminal Syn unique domain. β-finger via its binding groove[5].
3D Structures of syntrophin
Updated on 18-August-2024
References
- ↑ Bhat HF, Adams ME, Khanday FA. Syntrophin proteins as Santa Claus: role(s) in cell signal transduction. Cell Mol Life Sci. 2013 Jul;70(14):2533-54. doi: 10.1007/s00018-012-1233-9. Epub , 2012 Dec 21. PMID:23263165 doi:http://dx.doi.org/10.1007/s00018-012-1233-9
- ↑ Wu G, Ai T, Kim JJ, Mohapatra B, Xi Y, Li Z, Abbasi S, Purevjav E, Samani K, Ackerman MJ, Qi M, Moss AJ, Shimizu W, Towbin JA, Cheng J, Vatta M. alpha-1-syntrophin mutation and the long-QT syndrome: a disease of sodium channel disruption. Circ Arrhythm Electrophysiol. 2008 Aug;1(3):193-201. doi:, 10.1161/CIRCEP.108.769224. PMID:19684871 doi:http://dx.doi.org/10.1161/CIRCEP.108.769224
- ↑ Liang Y, Wang B, Chen S, Ye Z, Chai X, Li R, Li X, Kong G, Li Y, Zhang X, Che Z, Xie Q, Lian J, Lin B, Zhang X, Huang X, Huang W, Qiu X, Zeng J. Beta-1 syntrophin (SNTB1) regulates colorectal cancer progression and stemness via regulation of the Wnt/β-catenin signaling pathway. Ann Transl Med. 2021 Jun;9(12):1016. PMID:34277816 doi:10.21037/atm-21-2700
- ↑ Wu G, Ai T, Kim JJ, Mohapatra B, Xi Y, Li Z, Abbasi S, Purevjav E, Samani K, Ackerman MJ, Qi M, Moss AJ, Shimizu W, Towbin JA, Cheng J, Vatta M. alpha-1-syntrophin mutation and the long-QT syndrome: a disease of sodium channel disruption. Circ Arrhythm Electrophysiol. 2008 Aug;1(3):193-201. doi:, 10.1161/CIRCEP.108.769224. PMID:19684871 doi:http://dx.doi.org/10.1161/CIRCEP.108.769224
- ↑ Hillier BJ, Christopherson KS, Prehoda KE, Bredt DS, Lim WA. Unexpected modes of PDZ domain scaffolding revealed by structure of nNOS-syntrophin complex. Science. 1999 Apr 30;284(5415):812-5. PMID:10221915
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