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Gene Review

SNTA1  -  syntrophin, alpha 1

Homo sapiens

Synonyms: 59 kDa dystrophin-associated protein A1 acidic component 1, Alpha-1-syntrophin, LQT12, Pro-TGF-alpha cytoplasmic domain-interacting protein 1, SNT1, ...
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Disease relevance of SNTA1


High impact information on SNTA1


Biological context of SNTA1


Anatomical context of SNTA1


Associations of SNTA1 with chemical compounds

  • Understanding the assembly of this complex at the developing synapse led us to investigate, in Torpedo electrocyte, the intracellular routing and the targeting of several of its components, including dystroglycan, syntrophin, dystrophin and dystrobrevin [20].
  • Alpha1-syntrophin, a scaffolding adapter and modular protein, is a cytoplasmic component of the dystrophin glycoprotein complex [21].

Physical interactions of SNTA1


Enzymatic interactions of SNTA1

  • The observation that a monoclonal antibody directed against epitopes within exons 73-74 (MANEX7374A) failed to detect this 58 kDa protein provides definitive evidence that Dp71 big up tri, open(110) is derived from Dp71 transcripts deleted for the syntrophin-binding domain [15].

Other interactions of SNTA1


Analytical, diagnostic and therapeutic context of SNTA1


  1. Actinin-associated LIM protein: identification of a domain interaction between PDZ and spectrin-like repeat motifs. Xia, H., Winokur, S.T., Kuo, W.L., Altherr, M.R., Bredt, D.S. J. Cell Biol. (1997) [Pubmed]
  2. The differential diagnosis of the human dystrophinopathies and related disorders. Kakulas, B.A. Curr. Opin. Neurol. (1996) [Pubmed]
  3. C-terminal-truncated microdystrophin recruits dystrobrevin and syntrophin to the dystrophin-associated glycoprotein complex and reduces muscular dystrophy in symptomatic utrophin/dystrophin double-knockout mice. Yue, Y., Liu, M., Duan, D. Mol. Ther. (2006) [Pubmed]
  4. Cyclic peptides as non-carboxyl-terminal ligands of syntrophin PDZ domains. Gee, S.H., Sekely, S.A., Lombardo, C., Kurakin, A., Froehner, S.C., Kay, B.K. J. Biol. Chem. (1998) [Pubmed]
  5. Comparison of triflusal and aspirin for prevention of vascular events in patients after cerebral infarction: the TACIP Study: a randomized, double-blind, multicenter trial. Matías-Guiu, J., Ferro, J.M., Alvarez-Sabín, J., Torres, F., Jiménez, M.D., Lago, A., Melo, T. Stroke (2003) [Pubmed]
  6. Mammalian alpha 1- and beta 1-syntrophin bind to the alternative splice-prone region of the dystrophin COOH terminus. Suzuki, A., Yoshida, M., Ozawa, E. J. Cell Biol. (1995) [Pubmed]
  7. The sarcolemmal calcium pump, alpha-1 syntrophin, and neuronal nitric-oxide synthase are parts of a macromolecular protein complex. Williams, J.C., Armesilla, A.L., Mohamed, T.M., Hagarty, C.L., McIntyre, F.H., Schomburg, S., Zaki, A.O., Oceandy, D., Cartwright, E.J., Buch, M.H., Emerson, M., Neyses, L. J. Biol. Chem. (2006) [Pubmed]
  8. Syntrophins Regulate {alpha}1D-Adrenergic Receptors through a PDZ Domain-mediated Interaction. Chen, Z., Hague, C., Hall, R.A., Minneman, K.P. J. Biol. Chem. (2006) [Pubmed]
  9. Protein trafficking and anchoring complexes revealed by proteomic analysis of inward rectifier potassium channel (Kir2.x)-associated proteins. Leonoudakis, D., Conti, L.R., Anderson, S., Radeke, C.M., McGuire, L.M., Adams, M.E., Froehner, S.C., Yates, J.R., Vandenberg, C.A. J. Biol. Chem. (2004) [Pubmed]
  10. Syntrophin gamma 2 regulates SCN5A gating by a PDZ domain-mediated interaction. Ou, Y., Strege, P., Miller, S.M., Makielski, J., Ackerman, M., Gibbons, S.J., Farrugia, G. J. Biol. Chem. (2003) [Pubmed]
  11. Alternative splicing of dystrobrevin regulates the stoichiometry of syntrophin binding to the dystrophin protein complex. Newey, S.E., Benson, M.A., Ponting, C.P., Davies, K.E., Blake, D.J. Curr. Biol. (2000) [Pubmed]
  12. Interaction of gamma 1-syntrophin with diacylglycerol kinase-zeta. Regulation of nuclear localization by PDZ interactions. Hogan, A., Shepherd, L., Chabot, J., Quenneville, S., Prescott, S.M., Topham, M.K., Gee, S.H. J. Biol. Chem. (2001) [Pubmed]
  13. Stress-activated protein kinase-3 interacts with the PDZ domain of alpha1-syntrophin. A mechanism for specific substrate recognition. Hasegawa, M., Cuenda, A., Spillantini, M.G., Thomas, G.M., Buée-Scherrer, V., Cohen, P., Goedert, M. J. Biol. Chem. (1999) [Pubmed]
  14. Alpha1-syntrophin has distinct binding sites for actin and calmodulin. Iwata, Y., Pan, Y., Yoshida, T., Hanada, H., Shigekawa, M. FEBS Lett. (1998) [Pubmed]
  15. Expression and synthesis of alternatively spliced variants of Dp71 in adult human brain. Austin, R.C., Morris, G.E., Howard, P.L., Klamut, H.J., Ray, P.N. Neuromuscul. Disord. (2000) [Pubmed]
  16. Association of utrophin and multiple dystrophin short forms with the mammalian M(r) 58,000 dystrophin-associated protein (syntrophin). Kramarcy, N.R., Vidal, A., Froehner, S.C., Sealock, R. J. Biol. Chem. (1994) [Pubmed]
  17. Multiple effector domains within SNT1 coordinate ERK activation and neuronal differentiation of PC12 cells. Xu, H., Goldfarb, M. J. Biol. Chem. (2001) [Pubmed]
  18. Regulation of brain aquaporins. Gunnarson, E., Zelenina, M., Aperia, A. Neuroscience (2004) [Pubmed]
  19. Ultrastructural localization of alpha 1-syntrophin and neuronal nitric oxide synthase in normal skeletal myofiber, and their relation to each other and to dystrophin. Wakayama, Y., Inoue, M., Murahashi, M., Shibuya, S., Jimi, T., Kojima, H., Oniki, H. Acta Neuropathol. (1997) [Pubmed]
  20. Differential targeting of components of the dystrophin complex to the postsynaptic membrane. Marchand, S., Stetzkowski-Marden, F., Cartaud, J. Eur. J. Neurosci. (2001) [Pubmed]
  21. Altered alpha1-syntrophin expression in myofibers with Duchenne and Fukuyama muscular dystrophies. Wakayama, Y., Inoue, M., Kojima, H., Jimi, T., Yamashita, S., Kumagai, T., Shibuya, S., Hara, H., Oniki, H. Histol. Histopathol. (2006) [Pubmed]
  22. Syntrophin binds to an alternatively spliced exon of dystrophin. Ahn, A.H., Kunkel, L.M. J. Cell Biol. (1995) [Pubmed]
  23. Regulation of capacitative calcium entries by alpha1-syntrophin: association of TRPC1 with dystrophin complex and the PDZ domain of alpha1-syntrophin. Vandebrouck, A., Sabourin, J., Rivet, J., Balghi, H., Sebille, S., Kitzis, A., Raymond, G., Cognard, C., Bourmeyster, N., Constantin, B. FASEB J. (2007) [Pubmed]
  24. Characterization of the dystrophin-syntrophin interaction using the two-hybrid system in yeast. Castelló, A., Brochériou, V., Chafey, P., Kahn, A., Gilgenkrantz, H. FEBS Lett. (1996) [Pubmed]
  25. A 5' dystrophin duplication mutation causes membrane deficiency of alpha-dystroglycan in a family with X-linked cardiomyopathy. Bies, R.D., Maeda, M., Roberds, S.L., Holder, E., Bohlmeyer, T., Young, J.B., Campbell, K.P. J. Mol. Cell. Cardiol. (1997) [Pubmed]
  26. A novel form of familial congenital muscular dystrophy in two adolescents. Salih, M.A., Al Rayess, M., Cutshall, S., Urtizberea, J.A., Al-Turaiki, M.H., Ozo, C.O., Straub, V., Akbar, M., Abid, M., Andeejani, A., Campbell, K.P. Neuropediatrics. (1998) [Pubmed]
  27. Formation of nNOS/PSD-95 PDZ dimer requires a preformed beta-finger structure from the nNOS PDZ domain. Tochio, H., Mok, Y.K., Zhang, Q., Kan, H.M., Bredt, D.S., Zhang, M. J. Mol. Biol. (2000) [Pubmed]
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