title: Novel acyl-CoA synthetase in adrenoleukodystrophy target tissues.
abstract: 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.
authors: Muscle Differentiation Group, Mitsubishi Kagaku Institute of Life Sciences, 11 Minamiooya, Machida, Tokyo 194-8511, Japan.
authors: A
authors: A
authors: Moriya-Sato
authors: A
authors: A
authors: Hida
authors: M
authors: M
authors: Inagawa-Ogashiwa
authors: M R
authors: MR
authors: Wada
authors: K
authors: K
authors: Sugiyama
authors: J
authors: J
authors: Shimizu
authors: T
authors: T
authors: Yabuki
authors: Y
authors: Y
authors: Seyama
authors: N
authors: N
authors: Hashimoto
mesh_terms: D000326:Adrenoleukodystrophy / Q000201:enzymology* / Q000235:genetics; D000595:Amino Acid Sequence; D000818:Animals; D001483:Base Sequence; D019556:COS Cells; D003001:Cloning, Molecular; D003066:Coenzyme A Ligases / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D018076:DNA, Complementary; D008040:Genetic Linkage; D006801:Humans; D051379:Mice; D008969:Molecular Sequence Data; D017386:Sequence Homology, Amino Acid; D014960:X Chromosome
chemical_list: D018076:DNA, Complementary; D003066:Coenzyme A Ligases; C418138:lipidosin