The sarcoglycan-sarcospan complex localization in mouse retina is independent from dystrophins.
The sarcoglycan-sarcospan (SG-SSPN) complex is part of the dystrophin-glycoprotein complex that has been extensively characterized in muscle. To establish the framework for functional studies of sarcoglycans in retina here, we quantified sarcoglycans mRNA levels with real-time reverse transcriptase-polymerase chain reaction (RT-PCR) and performed immunohistochemistry to determine their cellular and subcellular distribution. We showed that the beta-, delta-, gamma-, epsilon-sarcoglycans and sarcospan are expressed in mouse retina. They are localized predominantly in the outer and the inner limiting membranes, probably in the Müller cells and also in the ganglion cells axons where the expression of dystrophins have never been reported. We also investigated the status of the sarcoglycans in the retina of mdx(3cv) mutant mice for all Duchene Muscular Dystrophy (DMD) gene products. The absence of dystrophin did not produce any change in the sarcoglycan-sarcospan components expression and distribution.
INSERM U-592, Laboratoire de Physiopathologie Cellulaire et Moléculaire de la Rétine, Hôpital Saint Antoine, Bat Kourilsky 184 rue du Fbg St Antoine, F-75571 Paris Cedex, France.
Patrice
P
Fort
Francisco-Javier
FJ
Estrada
Agnès
A
Bordais
Dominique
D
Mornet
José-Alain
JA
Sahel
Serge
S
Picaud
Haydeé Rosas
HR
Vargas
Ramón M
RM
Coral-Vázquez
Alvaro
A
Rendon
D000818:Animals; D002352:Carrier Proteins / Q000378:metabolism*; D002478:Cells, Cultured; D016189:Dystrophin / Q000378:metabolism*; D005786:Gene Expression Regulation / Q000502:physiology; D005970:Glutamate Synthase / Q000378:metabolism; D007150:Immunohistochemistry / Q000379:methods; D046911:Macromolecular Substances / Q000378:metabolism; D008565:Membrane Proteins / Q000378:metabolism*; D051379:Mice; D008810:Mice, Inbred C57BL; D018101:Mice, Inbred mdx; D009363:Neoplasm Proteins / Q000378:metabolism*; D016900:Neurofilament Proteins / Q000378:metabolism; D009457:Neuroglia / Q000378:metabolism; D012333:RNA, Messenger / Q000096:biosynthesis; D012160:Retina / Q000166:cytology / Q000378:metabolism*; D012165:Retinal Ganglion Cells / Q000378:metabolism; D020133:Reverse Transcriptase Polymerase Chain Reaction / Q000379:methods; D049031:Sarcoglycans / Q000378:metabolism*
D002352:Carrier Proteins; D016189:Dystrophin; D046911:Macromolecular Substances; D008565:Membrane Proteins; D009363:Neoplasm Proteins; D016900:Neurofilament Proteins; D012333:RNA, Messenger; D049031:Sarcoglycans; C491632:Sspn protein, mouse; C066858:neurofilament protein NF 68; D005970:Glutamate Synthase