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Syntrophin information


The syntrophins are a family of five 60-kiloDalton proteins that are associated with dystrophin, the protein associated with Duchenne muscular dystrophy and Becker muscular dystrophy. The name comes from the Greek word syntrophos, meaning "companion." The five syntrophins are encoded by separate genes and are termed α, β1, β2, γ1, and γ2. Syntrophin was first identified as a dystrophin-associated protein present in the Torpedo electric organ (originally called "58K protein"). Subsequently, α-syntrophin was shown to be the predominant isoform in skeletal muscle where it is localized on the sarcolemma and enriched at the neuromuscular junction. The β-syntrophins and γ2-syntrophin are also present in skeletal muscle but also are in most other tissues. The expression of γ1-syntrophin is mostly confined to brain. The syntrophins are adaptor proteins that use their multiple protein interaction domains (two pleckstrin homology domains and a PDZ domain) to localize a variety of signaling proteins (kinases, ion channels, water channels, nitric oxide synthase) to specific intracellular locations. α-Syntrophin binds to nNOS in the dystrophin-associated glycoprotein complex in skeletal muscle cells. There it produces NO upon muscle contraction leading to dilation of the arteries in the local area.[1]

  1. ^ http://jcs.biologists.org/content/117/13/2627.full.pdf [bare URL PDF]

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Syntrophin

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The syntrophins are a family of five 60-kiloDalton proteins that are associated with dystrophin, the protein associated with Duchenne muscular dystrophy...

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Dystrophin

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Chamberlain JS, Campbell KP (March 1995). "Identification of alpha-syntrophin binding to syntrophin triplet, dystrophin, and utrophin". The Journal of Biological...

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Stanley Froehner

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density protein PSD-95 and α1-syntrophin mediated by PDZ domains, at 1662 times according to Google Scholar. An α-syntrophin-dependent pool of AQP4 in astroglial...

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SNTB1

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Beta-1-syntrophin is a protein that in humans is encoded by the SNTB1 gene. Dystrophin is a large, rod-like cytoskeletal protein found at the inner surface...

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Long QT syndrome

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associated protein which interacts with KV7.1. LQT12 601017 SNTA1 Encodes syntrophin-α1. Mutations in this gene may increase the late sodium current INa. LQT13...

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SNTB2

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Beta-2-syntrophin is a protein that in humans is encoded by the SNTB2 gene. Dystrophin is a large, rod-like cytoskeletal protein found at the inner surface...

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Signal transducing adaptor protein

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SH2D3A -SH2 domain containing 3A SH2D3C – SH2 domain containing 3C SNTA1 – Syntrophin, alpha 1 SHB – Src homology 2 domain containing adaptor protein B SLC4A1AP...

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ACTA2

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Iwata Y, Pan Y, Yoshida T, Hanada H, Shigekawa M (February 1998). "Alpha1-syntrophin has distinct binding sites for actin and calmodulin". FEBS Letters. 423...

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Pleckstrin homology domain

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kinesin-like protein unc-104 (see InterPro: IPR001752), spectrin beta-chain, syntrophin (2 PH domains), and S. cerevisiae nuclear migration protein NUM1. Oxysterol-binding...

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NOS1

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oxide synthase with the postsynaptic density protein PSD-95 and alpha1-syntrophin mediated by PDZ domains". Cell. 84 (5): 757–67. doi:10.1016/S0092-8674(00)81053-3...

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SNTG1

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Gamma-1-syntrophin is a protein that in humans is encoded by the SNTG1 gene. The protein encoded by this gene is a member of the syntrophin family. Syntrophins...

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Stress granule

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Containing 1 SNRPF SNRPF Small nuclear ribonucleoprotein F SNTB2 SNTB2 Beta-2-syntrophin SOGA3 SOGA3 SOGA Family Member 3 SORBS1 SORBS1 Sorbin and SH3 domain-containing...

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DLG4

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oxide synthase with the postsynaptic density protein PSD-95 and alpha1-syntrophin mediated by PDZ domains". Cell. 84 (5): 757–67. doi:10.1016/S0092-8674(00)81053-3...

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Aldehyde oxidase 1

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carboxyterminus of the ATP-binding cassette transporter A1 interacts with a beta2-syntrophin/utrophin complex". Biochem. Biophys. Res. Commun. 293 (2): 759–65. doi:10...

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Nitric oxide synthase

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PMID 8619882. Ponting CP, Phillips C (March 1995). "DHR domains in syntrophins, neuronal NO synthases and other intracellular proteins". Trends Biochem...

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SCN1A

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could conduct the test on site. Nav1.1 has been shown to interact with syntrophin, alpha 1. Sodium channel GRCh38: Ensembl release 89: ENSG00000144285 –...

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PDZ domain

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with Shaker-type K+ channels. A PDZ interaction between PSD-95, nNOS and syntrophin is mediated by the second PDZ domain. The third and final PDZ domain links...

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ABCA1

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carboxyterminus of the ATP-binding cassette transporter A1 interacts with a beta2-syntrophin/utrophin complex". Biochemical and Biophysical Research Communications...

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Dystrobrevin

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and brain, and forms complexes with dystrophin-associated proteins and syntrophin in liver and brain. In the brain, β-dystrobrevin associates with dystrophin...

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MAST1

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Yap CC, Yamazaki Y, et al. (2003). "Sast124, a novel splice variant of syntrophin-associated serine/threonine kinase (SAST), is specifically localized in...

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SNTG2

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SNTG2 is a syntrophin gene. v t e...

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Costamere

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and lambda sarcoglycan), sarcospan, dystroglycan (alpha and beta), and syntrophin. These proteins are thought to play an important role in maintaining the...

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ARHGEF12

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transporter A1 and associated binding proteins reveals the importance of beta1-syntrophin in cholesterol efflux". The Journal of Biological Chemistry. 280 (47):...

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MPDZ

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transporter A1 and associated binding proteins reveals the importance of beta1-syntrophin in cholesterol efflux". J. Biol. Chem. 280 (47): 39653–64. doi:10.1074/jbc...

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Utrophin

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doi:10.1006/excr.1995.1360. PMID 7589238. Ahn AH, Kunkel LM (1995). "Syntrophin binds to an alternatively spliced exon of dystrophin". J. Cell Biol. 128...

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DGKZ

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Hogan A, Shepherd L, Chabot J, et al. (2001). "Interaction of gamma 1-syntrophin with diacylglycerol kinase-zeta. Regulation of nuclear localization by...

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