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SNTB1  -  syntrophin, beta 1 (dystrophin-associated...

Homo sapiens

Synonyms: 59 kDa dystrophin-associated protein A1 basic component 1, 59-DAP, A1B, BSYN2, Beta-1-syntrophin, ...
 
 
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Disease relevance of SNTB1

  • Brazilian family with pure autosomal dominant spastic paraplegia maps to 8q: analysis of muscle beta 1 syntrophin [1].
  • Analyses of beta-1 syntrophin, syndecan 2 and gem GTPase as candidates for chicken muscular dystrophy [2].
  • A statistically significant difference did emerge when the A+AB excess in thrombosis was contrasted with the O+B excess in haemorrhage, suggesting that this difference might be accounted for the major A subgroup (A1) and, consequently, A1B [3].
  • The recombinant A1B protein, expressed in Escherichia coli, shows the same electrophoretic mobility and charge as the cellular one [4].
  • Chromatographic evidence indicated that the three trisaccharide structures were also present in other A1, A2, A1B and A2B ovarian cyst glycoproteins and in A1 and A2 salivary glycoproteins [5].
 

High impact information on SNTB1

  • We expressed the cDNA of human beta 1-syntrophin and the COOH terminus of human dystrophin in reticulocyte lysates using an in vitro transcription/translation system [6].
  • The C-terminal Gly-rich domain is necessary for alternative splicing activity, for stable RNA binding and for optimal RNA annealing activity. hnRNP A1B, which is an alternatively spliced isoform of hnRNP A1 with a longer Gly-rich domain, binds more strongly to pre-mRNA but has only limited alternative splicing activity [7].
  • In vitro translation of the mRNAs selected by hybridization with A1 cDNA produced two proteins of 34 and 38 kd; Northern blot analysis of poly(A)+ RNA from HeLa cells revealed that the abundance of the A1B mRNA was approximately 5% that of A1 [4].
  • At 2030 in an A1B future, increased O(3) precursor emissions enhance surface sulfate over India and China by up to 20% because of increased levels of OH and gas-phase SO(2) oxidation rates and add up to 20% to the direct sulfate forcing for that region relative to the present day [8].
  • A comparison of our human cDNA sequence with the GenBank expressed sequence tag (EST) data base has identified a relative from human skeletal muscle, EST25263, which is probably a human homologue of the published mouse syntrophin 2 [9].
 

Biological context of SNTB1

 

Anatomical context of SNTB1

  • A novel interaction with the PDZ protein beta1-syntrophin was identified using this approach, and this interaction was confirmed in human THP-1 macrophages and in mouse liver [11].
  • Affinity-purified antibodies against rabbit 59-1 DAP fusion proteins only recognize the lowest band of the 59-DAP triplet in skeletal muscle sarcolemma and isolated dystrophin-glycoprotein complex [14].
  • We conclude that biglycan is important for the maintenance of muscle cell integrity and plays a direct role in regulating the expression and sarcolemmal localization of the intracellular signaling proteins dystrobrevin-1 and -2, alpha- and beta1-syntrophin and nNOS [15].
  • The existence of atypical blood group galactosyltransferase which causes an expression of A2 character in A1B red blood cells [16].
  • Spatio-temporal expression of the trans-acting splicing factors SF2/ASF and heterogeneous ribonuclear proteins A1/A1B in the myometrium of the pregnant human uterus: a molecular mechanism for regulating regional protein isoform expression in vivo [13].
 

Associations of SNTB1 with chemical compounds

  • Aminoisobutyric acid (A1B), a specific substrate of the A system of amino acid transport, at a concentration between 1 and 50 mM had no significant effect on melphalan uptake at 3 min in any of the human cells studied except those of patient C [17].
  • Alpha 1-beta glycoprotein (A1B) was purified to homogeneity from human plasma using a three-step procedure involving pseudo-ligand affinity chromatography on immobilized Cibacron Blue 3-GA, gel filtration chromatography on Sephadex G-200 and ion-exchange chromatography on DEAE Affigel Blue. The overall yield of the combined techniques was 31% [18].
  • Normal human erythrocytes of blood groups A1, A2, B and O, and En (a-) erythrocytes lacking glycophorin A, but with A1B-activity, were surface-labeled with tritiated sodium borohydride after oxidation of terminal galactosyl and N-acetylgalactosaminyl residues with galactose oxidase [19].
 

Physical interactions of SNTB1

 

Analytical, diagnostic and therapeutic context of SNTB1

  • Immunohistochemical and Western Blot (WB) studies showed that the distribution, expression, and apparent molecular weight of the beta 1 syntrophin protein were comparable to those of normal control individuals [1].
  • ABO-incompatible kidney transplantation of an 8-yr-old girl with donor/recipient-constellation A1B/B [21].
  • Protein content and enzymatic and allergenic properties of all venoms studied were determined by dye stain reagent, hyaluronidase and phospholipase A1B enzyme activities, and radioallergosorbent test inhibition studies, respectively [22].
  • Several Black AB subjects classified as non-A1 by the agglutination test were identified as A1B and AintB on the enzyme basis [23].
  • This case report describes the transplantation of a kidney from an A1B donor to a recipient who was blood group A2B [24].

References

  1. Brazilian family with pure autosomal dominant spastic paraplegia maps to 8q: analysis of muscle beta 1 syntrophin. Rocco, P., Vainzof, M., Froehner, S.C., Peters, M.F., Marie, S.K., Passos-Bueno, M.R., Zatz, M. Am. J. Med. Genet. (2000) [Pubmed]
  2. Analyses of beta-1 syntrophin, syndecan 2 and gem GTPase as candidates for chicken muscular dystrophy. Yoshizawa, K., Inaba, K., Mannen, H., Kikuchi, T., Mizutani, M., Tsuji, S. Exp. Anim. (2003) [Pubmed]
  3. Cerebral thrombosis, cerebral haemorrhage, and ABO blood-groups. Ionescu, D.A., Marcu, I., Bicescu, E. Lancet (1976) [Pubmed]
  4. Alternative splicing in the human gene for the core protein A1 generates another hnRNP protein. Buvoli, M., Cobianchi, F., Bestagno, M.G., Mangiarotti, A., Bassi, M.T., Biamonti, G., Riva, S. EMBO J. (1990) [Pubmed]
  5. A-active trisaccharides isolated from A1 and A2 blood-group-specific glycoproteins. Donald, A.S. Eur. J. Biochem. (1981) [Pubmed]
  6. Syntrophin binds to an alternatively spliced exon of dystrophin. Ahn, A.H., Kunkel, L.M. J. Cell Biol. (1995) [Pubmed]
  7. Function of conserved domains of hnRNP A1 and other hnRNP A/B proteins. Mayeda, A., Munroe, S.H., Cáceres, J.F., Krainer, A.R. EMBO J. (1994) [Pubmed]
  8. Cross influences of ozone and sulfate precursor emissions changes on air quality and climate. Unger, N., Shindell, D.T., Koch, D.M., Streets, D.G. Proc. Natl. Acad. Sci. U.S.A. (2006) [Pubmed]
  9. Cloning of human basic A1, a distinct 59-kDa dystrophin-associated protein encoded on chromosome 8q23-24. Ahn, A.H., Yoshida, M., Anderson, M.S., Feener, C.A., Selig, S., Hagiwara, Y., Ozawa, E., Kunkel, L.M. Proc. Natl. Acad. Sci. U.S.A. (1994) [Pubmed]
  10. The syntrophin-dystrobrevin subcomplex in human neuromuscular disorders. Compton, A.G., Cooper, S.T., Hill, P.M., Yang, N., Froehner, S.C., North, K.N. J. Neuropathol. Exp. Neurol. (2005) [Pubmed]
  11. Purification of ATP-binding cassette transporter A1 and associated binding proteins reveals the importance of beta1-syntrophin in cholesterol efflux. Okuhira, K., Fitzgerald, M.L., Sarracino, D.A., Manning, J.J., Bell, S.A., Goss, J.L., Freeman, M.W. J. Biol. Chem. (2005) [Pubmed]
  12. Distinct sets of adjacent heterogeneous nuclear ribonucleoprotein (hnRNP) A1/A2 binding sites control 5' splice site selection in the hnRNP A1 mRNA precursor. Hutchison, S., LeBel, C., Blanchette, M., Chabot, B. J. Biol. Chem. (2002) [Pubmed]
  13. Spatio-temporal expression of the trans-acting splicing factors SF2/ASF and heterogeneous ribonuclear proteins A1/A1B in the myometrium of the pregnant human uterus: a molecular mechanism for regulating regional protein isoform expression in vivo. Pollard, A.J., Sparey, C., Robson, S.C., Krainer, A.R., Europe-Finner, G.N. J. Clin. Endocrinol. Metab. (2000) [Pubmed]
  14. Heterogeneity of the 59-kDa dystrophin-associated protein revealed by cDNA cloning and expression. Yang, B., Ibraghimov-Beskrovnaya, O., Moomaw, C.R., Slaughter, C.A., Campbell, K.P. J. Biol. Chem. (1994) [Pubmed]
  15. Biglycan regulates the expression and sarcolemmal localization of dystrobrevin, syntrophin, and nNOS. Mercado, M.L., Amenta, A.R., Hagiwara, H., Rafii, M.S., Lechner, B.E., Owens, R.T., McQuillan, D.J., Froehner, S.C., Fallon, J.R. FASEB J. (2006) [Pubmed]
  16. The existence of atypical blood group galactosyltransferase which causes an expression of A2 character in A1B red blood cells. Yoshida, A. Am. J. Hum. Genet. (1983) [Pubmed]
  17. Effects of amino acids on the transport and cytotoxicity of melphalan by human bone marrow cells and human tumor cells. Dufour, M., Panasci, L.C., St Germain, J., Boulet, L. Cancer Chemother. Pharmacol. (1985) [Pubmed]
  18. Purification of human alpha 1-beta glycoprotein and study of its microheterogeneity and molecular variants. Miribel, L., Arnaud, P. J. Chromatogr. (1987) [Pubmed]
  19. Identification of blood group A-active glycoproteins in the human erythrocyte membrane. Karhi, K.K., Gahmberg, C.G. Biochim. Biophys. Acta (1980) [Pubmed]
  20. 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]
  21. ABO-incompatible kidney transplantation of an 8-yr-old girl with donor/recipient-constellation A1B/B. Ahlenstiel, T., Offner, G., Strehlau, J., Pape, L., Froede, K., Ehrich, J.H., Schwarz, A., Heuft, H.G., Klempnauer, J. Xenotransplantation (2006) [Pubmed]
  22. Comparison of Vespula germanica venoms obtained from different sources. Sanchez, F., Blanca, M., Miranda, A., Carmona, M.J., Garcia, J., Fernandez, J., Torres, M.J., Rondon, M.C., Juarez, C. Int. Arch. Allergy Immunol. (1994) [Pubmed]
  23. Uncertainty in identification of blood group A subtypes by agglutination test. Yoshida, A., Davé, V., Prchal, J. Hum. Hered. (1985) [Pubmed]
  24. Renal transplantation from a blood group A1B donor to an A2B recipient: a case report. Albornoz, G., Korb, S., Kolovich, R., Light, J.A. Am. J. Hematol. (1989) [Pubmed]
 
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