TY - JOUR
T1 - Identification of a fourth half ABC transporter in the human peroxisomal membrane
AU - Shani, Noam
AU - Jimenez-Sanchez, Gerardo
AU - Steel, Gary
AU - Dean, Michael
AU - Valle, David
N1 - Funding Information:
Computing resources were provided by the Frederick Biomedical Supercomputing Center. The content of this publication does not necessarily reflect the views or policies of the Department of Health and Human Services, nor does mention of trade names, commercial products or organizations imply endorsement by the U.S. Government. This work was supported in part by NIH grant 2P01HD10981 to the Kennedy Krieger Institute (NS). D.V. is an Investigator in The Howard Hughes Medical Institute. G.J.S. is supported in part by a fellowship from the Instituto de Investiga-ciones Biomedicas/DGAPA, UNAM.
PY - 1997/10
Y1 - 1997/10
N2 - 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 chromesome 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.
AB - 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 chromesome 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.
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U2 - 10.1093/hmg/6.11.1925
DO - 10.1093/hmg/6.11.1925
M3 - Article
C2 - 9302272
AN - SCOPUS:0030776037
SN - 0964-6906
VL - 6
SP - 1925
EP - 1931
JO - Human molecular genetics
JF - Human molecular genetics
IS - 11
ER -