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Human adrenoleukodystrophy protein and related peroxisomal ABC transporters interact with the peroxisomal assembly protein PEX19p

Gloeckner, CJ, Mayerhofer, PU, Landgraf, P, Muntau, AC, Holzinger, A, Gerber, JK, Kammerer, S, Adamski, J and Roscher, AA (2000) Human adrenoleukodystrophy protein and related peroxisomal ABC transporters interact with the peroxisomal assembly protein PEX19p Biochem Biophys Res Commun, 271. pp. 144-150.

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Abstract

Four ABC half transporters (ALDP, ALDRP, PMP70, and PMP69) have been identified in the mammalian peroxisomal membrane but no function has been unambiguously assigned to any of them. To date X-linked adrenoleukodystrophy (X-ALD) is the only human disease known to result from a defect of one of these ABC transporters, ALDP. Using the yeast two-hybrid system and in vitro GST pull-down assays, we identified the peroxin PEX19p as a novel interactor of ALDP, ALDRP, and PMP70. The cytosolic farnesylated protein PEX19p was previously shown to be involved in an early step of the peroxisomal biogenesis. The PEX19p interaction occurs in an internal N-terminal region of ALDP which we verified to be important for proper peroxisomal targeting of this protein. Farnesylated wild-type PEX19p and a farnesylation-deficient mutant PEX19p did not differ in their ability to bind to ALDP. Our data provide evidence that PEX19p is a cytosolic acceptor protein for the peroxisomal ABC transporters ALDP, PMP70, and ALDRP and might be involved in the intracellular sorting and trafficking of these proteins to the peroxisomal membrane.

Item Type: Article
Authors :
NameEmailORCID
Gloeckner, CJUNSPECIFIEDUNSPECIFIED
Mayerhofer, PUp.mayerhofer@surrey.ac.ukUNSPECIFIED
Landgraf, PUNSPECIFIEDUNSPECIFIED
Muntau, ACUNSPECIFIEDUNSPECIFIED
Holzinger, AUNSPECIFIEDUNSPECIFIED
Gerber, JKUNSPECIFIEDUNSPECIFIED
Kammerer, SUNSPECIFIEDUNSPECIFIED
Adamski, JUNSPECIFIEDUNSPECIFIED
Roscher, AAUNSPECIFIEDUNSPECIFIED
Date : 2000
Identification Number : https://doi.org/10.1006/bbrc.2000.2572
Uncontrolled Keywords : ATP-Binding Cassette Transporters/chemistry/*metabolism Adrenoleukodystrophy/metabolism Animals Brain/metabolism COS Cells DNA, Complementary/metabolism Gene Library Glutathione Transferase/metabolism Humans Membrane Proteins/chemistry/*metabolism Peroxisomes/chemistry/*metabolism Plasmids Protein Binding Protein Prenylation Protein Structure, Tertiary Proteins/chemistry/*metabolism Recombinant Fusion Proteins/metabolism Transfection Two-Hybrid System Techniques
Related URLs :
Depositing User : Symplectic Elements
Date Deposited : 17 May 2017 10:42
Last Modified : 17 May 2017 14:51
URI: http://epubs.surrey.ac.uk/id/eprint/828861

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