The photoreceptor rim protein is an ABC transporter encoded by the gene for recessive Stargardt's disease (ABCR)

被引:149
作者
Azarian, SM
Travis, GH
机构
[1] UNIV TEXAS,SW MED CTR,DEPT PSYCHIAT,DALLAS,TX 75235
[2] UNIV TEXAS,SW MED CTR,PROGRAM NEUROSCI,DALLAS,TX 75235
关键词
rim protein; Stargardt's disease; outer segment; macular degeneration; transporter; retinal degeneration;
D O I
10.1016/S0014-5793(97)00517-6
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
Rim protein (RmP) is a high-M-r membrane glycoprotein that has been localized to the rims of photoreceptor outer segment discs, but its molecular identity is unknown. Here, we describe the purification of RmP and present the sequence of its mRNA. RmP is a new member of the ATP-binding cassette (ABC) transporter superfamily. We show that RmP is expressed specifically in photoreceptors and predominantly in outer segments. Further, RmP is identical to the protein recently shown, to be affected in recessive Stargardt's disease. RmP is the first ABC transporter observed in photoreceptors and may play a role in the photoresponse. (C) 1997 Federation of European Biochemical Societies.
引用
收藏
页码:247 / 252
页数:6
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