Genetic evidence for a dystrophin-glycoprotein complex (DGC) in Caenorhabditis elegans

被引:47
作者
Grisoni, K [1 ]
Martin, E [1 ]
Gieseler, K [1 ]
Mariol, MC [1 ]
Ségalat, L [1 ]
机构
[1] Univ Lyon 1, CNRS, UMR 5534, CGMC, F-69100 Villeurbanne, France
关键词
dystrophin; sarcoglycan; dystroglycan; syntrophin; nematode;
D O I
10.1016/S0378-1119(02)00762-X
中图分类号
Q3 [遗传学];
学科分类号
071007 ; 090102 ;
摘要
Dystrophin is the product of the gene mutated in Duchenne muscular dystrophy (DMD). Neither the function of dystrophin nor the physiopathology of the disease have been clearly established so far. In mammals, the dystrophin-glycoprotein complex (DGC) includes dystrophin, as well as transmembrane and cytoplasmic proteins. Since Caenorhabditis elegans possesses a dystrophin-like gene (dys-1), we investigated whether homologues of the DGC members could also be found in the C. elegans genome. Conserved homologues were found for dystroglycan, delta/gamma-sarcoglyean and syntrophim. Divergent but related proteins were found for alpha- and beta-sarcoglycans. No sarcospan counterpart was found. The expression of the conserved homologues was inactivated using the RNA interference technique. Phenotypes similar to that of dys-1 were obtained, both in the wild-type background and in combination with other mutations. These results strongly suggest that a protein complex comprising functional analogies with the DGC exists in C. elegans. (C) 2002 Elsevier Science B.V. All rights reserved.
引用
收藏
页码:77 / 86
页数:10
相关论文
共 36 条
[1]   Absence of α-syntrophin leads to structurally aberrant neuromuscular synapses deficient in utrophin [J].
Adams, ME ;
Kramarcy, N ;
Krall, SP ;
Rossi, SG ;
Rotundo, RL ;
Sealock, R ;
Froehner, SC .
JOURNAL OF CELL BIOLOGY, 2000, 150 (06) :1385-1397
[2]   SYNTROPHIN BINDS TO AN ALTERNATIVELY SPLICED EXON OF DYSTROPHIN [J].
AHN, AH ;
KUNKEL, LM .
JOURNAL OF CELL BIOLOGY, 1995, 128 (03) :363-371
[3]   THE STRUCTURAL AND FUNCTIONAL DIVERSITY OF DYSTROPHIN [J].
AHN, AH ;
KUNKEL, LM .
NATURE GENETICS, 1993, 3 (04) :283-291
[4]  
ALTSCHUL SF, 1990, J MOL BIOL, V215, P403, DOI 10.1006/jmbi.1990.9999
[5]   Mutations in the Caenorhabditis elegans dystrophin-like gene dys-1 lead to hyperactivity and suggest a link with cholinergic transmission [J].
Bessou, C ;
Giugia, JB ;
Franks, CJ ;
Holden-Dye, L ;
Ségalat, L .
NEUROGENETICS, 1998, 2 (01) :61-72
[6]   The limb-girdle muscular dystrophies-multiple genes, multiple mechanisms [J].
Bushby, KMD .
HUMAN MOLECULAR GENETICS, 1999, 8 (10) :1875-1882
[7]   Assembly of the dystrophin-associated protein complex does not require the dystrophin COOH-terminal domain [J].
Crawford, GE ;
Faulkner, JA ;
Crosbie, RH ;
Campbell, KP ;
Froehner, SC ;
Chamberlain, JS .
JOURNAL OF CELL BIOLOGY, 2000, 150 (06) :1399-1409
[8]   Sarcospan, the 25-kDa transmembrane component of the dystrophin-glycoprotein complex [J].
Crosbie, RH ;
Heighway, J ;
Venzke, DP ;
Lee, JC ;
Campbell, KP .
JOURNAL OF BIOLOGICAL CHEMISTRY, 1997, 272 (50) :31221-31224
[9]   THE ALPHA-DYSTROGLYCAN-BETA-DYSTROGLYCAN COMPLEX - MEMBRANE ORGANIZATION AND RELATIONSHIP TO AN AGRIN RECEPTOR [J].
DEYST, KA ;
BOWE, MA ;
LESZYK, JD ;
FALLON, JR .
JOURNAL OF BIOLOGICAL CHEMISTRY, 1995, 270 (43) :25956-25959
[10]  
Duret L, 1996, COMPUT APPL BIOSCI, V12, P507