SNTB2 explained

Beta-2-syntrophin is a protein that in humans is encoded by the SNTB2 gene.^{[1] [2] [3]}

Function

Dystrophin is a large, rod-like cytoskeletal protein found at the inner surface of muscle fibers. Dystrophin is missing in Duchenne Muscular Dystrophy patients and is present in reduced amounts in Becker Muscular Dystrophy patients. The protein encoded by this gene is a peripheral membrane protein found associated with dystrophin and dystrophin-related proteins. This gene is a member of the syntrophin gene family, which contains at least two other structurally related genes.

Interactions

SNTB2 has been shown to interact with ABCA1.^[4]

Further reading

• Blake DJ . Dystrobrevin dynamics in muscle-cell signalling: a possible target for therapeutic intervention in Duchenne muscular dystrophy? . Neuromuscul. Disord. . 12 . S110–7 . 2002 . Suppl 1 . 12206805 . 10.1016/S0960-8966(02)00091-3 . 9188156 .
• Gee SH, Madhavan R, Levinson SR, Caldwell JH, Sealock R, Froehner SC . Interaction of muscle and brain sodium channels with multiple members of the syntrophin family of dystrophin-associated proteins . J. Neurosci. . 18 . 1 . 128–37 . 1998 . 9412493 . 10.1523/JNEUROSCI.18-01-00128.1998. 6793384 . 10.1.1.469.2092 .
• Lumeng C, Phelps S, Crawford GE, Walden PD, Barald K, Chamberlain JS . Interactions between beta 2-syntrophin and a family of microtubule-associated serine/threonine kinases . Nat. Neurosci. . 2 . 7 . 611–7 . 1999 . 10404183 . 10.1038/10165 . 20910888 .
• Garcia RA, Vasudevan K, Buonanno A . The neuregulin receptor ErbB-4 interacts with PDZ-containing proteins at neuronal synapses . Proc. Natl. Acad. Sci. U.S.A. . 97 . 7 . 3596–601 . 2000 . 10725395 . 16285 . 10.1073/pnas.070042497 . free .
• Adams ME, Kramarcy N, Krall SP, Rossi SG, Rotundo RL, Sealock R, Froehner SC . Absence of α-Syntrophin Leads to Structurally Aberrant Neuromuscular Synapses Deficient in Utrophin . J. Cell Biol. . 150 . 6 . 1385–98 . 2000 . 10995443 . 2150701 . 10.1083/jcb.150.6.1385 .
• Ort T, Maksimova E, Dirkx R, Kachinsky AM, Berghs S, Froehner SC, Solimena M . The receptor tyrosine phosphatase-like protein ICA512 binds the PDZ domains of beta2-syntrophin and nNOS in pancreatic beta-cells . Eur. J. Cell Biol. . 79 . 9 . 621–30 . 2001 . 11043403 . 10.1078/0171-9335-00095 .
• Marchand S, Stetzkowski-Marden F, Cartaud J . Differential targeting of components of the dystrophin complex to the postsynaptic membrane . Eur. J. Neurosci. . 13 . 2 . 221–9 . 2001 . 11168526 . 10.1046/j.1460-9568.2001.01373.x .
• Hogan A, Shepherd L, Chabot J, Quenneville S, Prescott SM, Topham MK, Gee SH . Interaction of gamma 1-syntrophin with diacylglycerol kinase-zeta. Regulation of nuclear localization by PDZ interactions . J. Biol. Chem. . 276 . 28 . 26526–33 . 2001 . 11352924 . 10.1074/jbc.M104156200 . free .
• Ort T, Voronov S, Guo J, Zawalich K, Froehner SC, Zawalich W, Solimena M . Dephosphorylation of β2-syntrophin and Ca2+/µ-calpain-mediated cleavage of ICA512 upon stimulation of insulin secretion . EMBO J. . 20 . 15 . 4013–23 . 2001 . 11483505 . 149140 . 10.1093/emboj/20.15.4013 .
• Buechler C, Boettcher A, Bared SM, Probst MC, Schmitz G . The carboxyterminus of the ATP-binding cassette transporter A1 interacts with a beta2-syntrophin/utrophin complex . Biochem. Biophys. Res. Commun. . 293 . 2 . 759–65 . 2002 . 12054535 . 10.1016/S0006-291X(02)00303-0 .
• Kutsenko AS, Gizatullin RZ, Al-Amin AN, Wang F, Kvasha SM, Podowski RM, Matushkin YG, Gyanchandani A, Muravenko OV, Levitsky VG, Kolchanov NA, Protopopov AI, Kashuba VI, Kisselev LL, Wasserman W, Wahlestedt C, Zabarovsky ER . NotI flanking sequences: a tool for gene discovery and verification of the human genome . Nucleic Acids Res. . 30 . 14 . 3163–70 . 2002 . 12136098 . 135748 . 10.1093/nar/gkf428 .
• Chagnon P, Michaud J, Mitchell G, Mercier J, Marion JF, Drouin E, Rasquin-Weber A, Hudson TJ, Richter A . A Missense Mutation (R565W) in Cirhin (FLJ14728) in North American Indian Childhood Cirrhosis . Am. J. Hum. Genet. . 71 . 6 . 1443–9 . 2003 . 12417987 . 378590 . 10.1086/344580 .
• Jones KJ, Compton AG, Yang N, Mills MA, Peters MF, Mowat D, Kunkel LM, Froehner SC, North KN . Deficiency of the syntrophins and alpha-dystrobrevin in patients with inherited myopathy . Neuromuscul. Disord. . 13 . 6 . 456–67 . 2003 . 12899872 . 10.1016/S0960-8966(03)00066-X . 53270107 .
• Hillman RT, Green RE, Brenner SE . An unappreciated role for RNA surveillance . Genome Biol. . 5 . 2 . R8 . 2005 . 14759258 . 395752 . 10.1186/gb-2004-5-2-r8 . free .
• Leonoudakis D, Conti LR, Anderson S, Radeke CM, McGuire LM, Adams ME, Froehner SC, Yates JR, Vandenberg CA . Protein trafficking and anchoring complexes revealed by proteomic analysis of inward rectifier potassium channel (Kir2.x)-associated proteins . J. Biol. Chem. . 279 . 21 . 22331–46 . 2004 . 15024025 . 10.1074/jbc.M400285200 . free .

Notes and References

1. Ahn AH, Freener CA, Gussoni E, Yoshida M, Ozawa E, Kunkel LM . The three human syntrophin genes are expressed in diverse tissues, have distinct chromosomal locations, and each bind to dystrophin and its relatives . J Biol Chem . 271 . 5 . 2724–30 . March 1996 . 8576247 . 10.1074/jbc.271.5.2724 . free .
2. Ahn AH, Yoshida M, Anderson MS, Feener CA, Selig S, Hagiwara Y, Ozawa E, Kunkel LM . Cloning of human basic A1, a distinct 59-kDa dystrophin-associated protein encoded on chromosome 8q23-24 . Proc Natl Acad Sci U S A . 91 . 10 . 4446–50 . June 1994 . 8183929 . 43802 . 10.1073/pnas.91.10.4446 . 1994PNAS...91.4446A . free .
3. Web site: Entrez Gene: SNTB2 syntrophin, beta 2 (dystrophin-associated protein A1, 59kDa, basic component 2).
4. Buechler C, Boettcher A, Bared SM, Probst MC, Schmitz G . The carboxyterminus of the ATP-binding cassette transporter A1 interacts with a beta2-syntrophin/utrophin complex . Biochem. Biophys. Res. Commun. . 293 . 2 . 759–65 . May 2002 . 12054535 . 10.1016/S0006-291X(02)00303-0 .