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Titolo:
Differential expression and developmental regulation of a novel alpha-dystrobrevin isoform in muscle
Autore:
Enigk, RE; Maimone, MM;
Indirizzi:
SUNY Hlth Sci Ctr, Dept Anat & Cell Biol, Syracuse, NY 13210 USA SUNY HlthSci Ctr Syracuse NY USA 13210 Cell Biol, Syracuse, NY 13210 USA
Titolo Testata:
GENE
fascicolo: 2, volume: 238, anno: 1999,
pagine: 479 - 488
SICI:
0378-1119(19991001)238:2<479:DEADRO>2.0.ZU;2-Q
Fonte:
ISI
Lingua:
ENG
Soggetto:
TORPEDO ELECTRIC ORGAN; NEUROMUSCULAR-JUNCTION; SKELETAL-MUSCLE; GENOMIC ORGANIZATION; POSTSYNAPTIC PROTEIN; BETA-DYSTROBREVIN; MOUSE MUSCLE; DYSTROPHIN; SYNTROPHIN; IDENTIFICATION;
Keywords:
alternative splicing; C2C12 cells; cDNA cloning; dystrophin associated protein; RT-PCR;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
29
Recensione:
Indirizzi per estratti:
Indirizzo: Maimone, MM SUNY Hlth Sci Ctr, Dept Anat & Cell Biol, 750 E Adams St, Syracuse, NY 13210 USA SUNY Hlth Sci Ctr 750 E Adams St Syracuse NY USA 13210 210 USA
Citazione:
R.E. Enigk e M.M. Maimone, "Differential expression and developmental regulation of a novel alpha-dystrobrevin isoform in muscle", GENE, 238(2), 1999, pp. 479-488

Abstract

alpha-Dystrobrevin is a dystrophin-related protein expressed primarily in skeletal muscle, heart, lung and brain. In skeletal muscle, alpha-dystrobrevin is a component of the dystrophin-associated glycoprotein complex and islocalized to the sarcolemma, presumably through interactions with dystrophin and utrophin. Alternative splicing of the alpha-dystrobrevin gene generates multiple isoforms which have been grouped into three major classes: alpha-DB1, alpha-DB2, and alpha-DB3. Various isoforms have been shown to interact with a variety of proteins; however, the physiological function of the alpha-dystrobrevins remains unknown. In the present study, we have cloned anovel alpha-dystrobrevin cDNA encoding a protein (referred to as alpha-DB2b) with a unique 11 amino acid C-terminal tail. Using RT-PCR with primers specific to the new isoform, we have characterized its expression in skeletal muscle, heart, and brain, and in differentiating C2C12 muscle cells. We show that alpha-DB2b is expressed in skeletal muscle, heart and brain, and that exons 12 and 13 are alternatively spliced in alpha-DB2b to generate at least three splice variants. The major alpha-DB2b splice variant expressed in adult skeletal muscle and heart contains exons 12 and 13, while in adultbrain, two alpha-DB2b splice variants are expressed at similar levels. This is consistent with the preferential expression of exons 12 and 13 in other alpha-dystrobrevin isoforms in skeletal muscle and heart. Similarly, in alpha-DB1 the first 21 nucleotides of exon 18 are preferentially expressed in skeletal muscle and heart relative to brain. We also show that the expression of alternatively spliced alpha-DB2b is developmentally regulated in muscle; during differentiation of C2C12 cells, alpha-DB2b expression switchesfrom an isoform lacking exons 12 and 13 to one containing them. We demonstrate similar developmental upregulation of exons 12, 13, and 18 in alpha-DB1 and of exons 12 and 13 in alpha-DB2a. Finally, we show that alpha-DB2b protein is expressed in adult skeletal muscle, suggesting that it has a functional role in adult muscle. Together, these data suggest that alternativelyspliced variants of the new alpha-dystrobrevin isoform, alpha-DB2b, are differentially expressed in various tissues and developmentally regulated during muscle cell differentiation in a fashion similar to that previously described for alpha-dystrobrevin isoforms. (C) 1999 Elsevier Science B.V. All rights reserved.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 05/12/20 alle ore 16:04:26