Localization of mRNAs for adrenoleukodystrophy and the 70 kDa peroxisomal (PMP70) proteins in the rat brain during post-natal development

Journal of Neuroscience Research
H PollardP Aubourg

Abstract

Adrenoleukodystrophy (ALD) is a genetic demyelinating disorder caused by the mutation of a gene encoding a 75-kDa peroxisomal protein (ALDP) that belongs to the superfamily of ATP binding casette (ABC) transporters. The PMP 70 gene codes for another peroxisomal ABC transporter that shows 38.5% amino acid identity with ALDP. ALDP and PMP70 have the structure of half transporter and could possibly heterodimerize to form a full transporter within the peroxisomal membrane. Using in situ hybridization histochemistry in rat brain, we demonstrate that ALD and PMP70 mRNAs have different spatial and temporal expression during postnatal development. Whereas expression of PMP 70 mRNA was low at birth and culminates between the 2nd and 3rd week in hippocampus and cerebellum, maximum expression of ALDP was found at birth in all brain areas and decreased thereafter. The absence of coordinated expression of ALD and PMP70 genes suggests therefore that ALD and PMP70 proteins are unlikely to function as exclusive and obligatory partners in the brain.

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Adrenoleukodystrophy

Adrenoleukodystrophy (ALD), the most frequent peroxisomal disorder, is an X-linked disorder caused by a defect in the metabolism of long chain fatty acids leading to demyelination, neurodegeneration, and death. Here is the latest research.