Novel Acyl-CoA Synthetase in Adrenoleukodystrophy Target Tissues

X-linked adrenoleukodystrophy (X-ALD) is a neurodegenerative disorder characterized by demyelination of white matter. The X-ALD gene product adrenoleukodystrophy protein (ALDP) is expressed broadly among various tissues. However, deficiency of functional ALDP exclusively impairs brain, adrenal gland...

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Bibliographic Details
Published in:Biochemical and biophysical research communications 2000-12, Vol.279 (1), p.62-68
Main Authors: Moriya-Sato, Ayako, Hida, Azumi, Inagawa-Ogashiwa, Masayo, Wada, Michiko R., Sugiyama, Kaoru, Shimizu, Junko, Yabuki, Tomoko, Seyama, Yousuke, Hashimoto, Naohiro
Format: Article
Language:English
Subjects:
Addison disease
adrenoleukodystrophy
Adrenoleukodystrophy - enzymology
Adrenoleukodystrophy - genetics
adrenomyeloneuropathy
Amino Acid Sequence
Animals
Base Sequence
Bubblegum
Cloning, Molecular
Coenzyme A Ligases - chemistry
Coenzyme A Ligases - genetics
Coenzyme A Ligases - metabolism
COS Cells
DNA, Complementary
Genetic Linkage
Humans
lipidosis
long-chain acyl-CoA
long-chain acyl-CoA synthetase
long-chain fatty acid
Mice
Molecular Sequence Data
Sequence Homology, Amino Acid
very-long-chain acyl-CoA
very-long-chain fatty acid
X Chromosome
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Summary:X-linked adrenoleukodystrophy (X-ALD) is a neurodegenerative disorder characterized by demyelination of white matter. The X-ALD gene product adrenoleukodystrophy protein (ALDP) is expressed broadly among various tissues. However, deficiency of functional ALDP exclusively impairs brain, adrenal gland, and testis. Thus, loss of ALDP function is assumed to involve inactivation of a putative mediating factor that functions in a tissue-specific manner. Here we cloned a mouse cDNA encoding a novel protein, Lipidosin, that possesses long-chain acyl-CoA synthetase (LCAS) activity. Lipidosin is expressed exclusively in mouse brain, adrenal gland, and testis, which are affected by X-ALD. LCAS activity of Lipidosin was diminished by mutation of conserved amino acids within the AMP-binding domain. Mutation of the Drosophila homologue of Lipidosin has been reported to cause neuronal degeneration. Thus, Lipidosin may mediate the link between ALDP dysfunction and the impairment of fatty acid metabolism in X-ALD.
ISSN:0006-291X
1090-2104
DOI:10.1006/bbrc.2000.3897