Identification of a Fourth Half Abc Transporter in the Human Peroxisomal Membrane

Author:

Shani, N.

Jimenezsanchez, G.

Steel, G.

Dean, M.

Valle, D.
Author Address

Valle D JOHNS HOPKINS UNIV SCH MED DEPT PEDIAT BALTIMORE, MD 21205 USA JOHNS HOPKINS UNIV SCH MED DEPT PEDIAT BALTIMORE, MD 21205 USA JOHNS HOPKINS UNIV SCH MED KENNEDY KRIEGER INST BALTIMORE, MD 21205 USA JOHNS HOPKINS UNIV SCH MED PREDOCTORAL TRAINING PROGRAM BALTIMORE, MD 21205 USA JOHNS HOPKINS UNIV SCH MED HOWARD HUGHES MED INST BALTIMORE, MD 21205 USA NATL CANC RES & DEV CTR FREDERICK, MD 21702 USA

Abstract:

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. [References: 43]

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