Identification of a fourth half ABC transporter in the human peroxisomal membrane

被引:100
作者
Shani, N
JimenezSanchez, G
Steel, G
Dean, M
Valle, D
机构
[1] JOHNS HOPKINS UNIV,SCH MED,DEPT PEDIAT,BALTIMORE,MD 21205
[2] JOHNS HOPKINS UNIV,SCH MED,KENNEDY KRIEGER INST,BALTIMORE,MD 21205
[3] JOHNS HOPKINS UNIV,SCH MED,PREDOCTORAL TRAINING PROGRAM,BALTIMORE,MD 21205
[4] JOHNS HOPKINS UNIV,SCH MED,HOWARD HUGHES MED INST,BALTIMORE,MD 21205
[5] NATL CANC RES & DEV CTR,FREDERICK,MD 21702
关键词
D O I
10.1093/hmg/6.11.1925
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
Three half ATP-binding cassette transporters (ALDP, ALDR, PMP70) are known to be present in the human peroxisome membrane, Mutations in the gene encoding ALDP cause X-linked adrenoleukodystrophy; the role of ALDR and PMP70 in human disease is unclear. We report the cloning and characterization of a fourth human gene encoding a peroxisomal half ABC transporter, The gene, designated P70R, maps to chromosome 14q24, encodes a 73 kDa transporter most similar to PMP70, and is expressed in all human tissues examined. Because half ABC transporters heterodimerize to form functional transporters, the identification of a fourth member of this family in the peroxisome membrane has implications for our understanding of mammalian peroxisomes and the genetic disorders of peroxisomal function.
引用
收藏
页码:1925 / 1931
页数:7
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