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Neurology 1999;52:614
© 1999 American Academy of Neurology
Articles

Adrenoleukodystrophy protein enhances association of very long-chain acyl-coenzyme A synthetase with the peroxisome

T. Yamada, MD, T. Taniwaki, MD, N. Shinnoh, A. Uchiyama, MD, N. Shimozawa, MD, Y. Ohyagi, MD, H. Asahara and J. Kira, MD

From the Department of Neurology (Drs. Yamada, Taniwaki, Ohyagi, and Kira, and N. Shinnoh and H. Asahara), Neurological Institute, Faculty of Medicine, Kyushu University, Fukuoka; and the Department of Pediatrics (Drs. Uchiyama and Shimozawa), Gifu University School of Medicine, Gifu, Japan.

Address correspondence and reprint requests to Dr. Takeshi Yamada, Department of Neurology, Neurological Institute, Faculty of Medicine, Kyushu University, Fukuoka 812-8582, Japan; e-mail: yamada{at}neuro.med.kyushu-u.ac.jp

OBJECTIVE: To clarify the function of adrenoleukodystrophy protein (ALDP) using our ALDP-deficient mice established by gene targeting.

BACKGROUND: X-linked adrenoleukodystrophy (ALD) is characterized biochemically by the accumulation of very long-chain fatty acids (VLCFA) in tissues and body fluids, and is caused by impairment of peroxisomal ß-oxidation. In ALD, very long-chain acyl-coenzyme A synthetase (VLACS), which is necessary for peroxisomal ß-oxidation, does not function.

METHODS: The ALDP-deficient mice and C57BL/6J mice were used. VLACS or ALDP were transiently expressed by lipofection in murine fibroblasts, and VLCFA ß-oxidation was assayed. Liver peroxisomes were purified by sequential centrifugations and a Nycodenz gradient centrifugation. The peroxisomal localization of VLACS was compared between the mutant and control mice using a Western blot analysis.

RESULTS: Impairment of VLCFA ß-oxidation in ALDP-deficient fibroblasts was not corrected by the additional expression of VLACS alone but was by the coexpression of VLACS and ALDP. Although the tissue-specific expression of VLACS was similar in ALDP-deficient and normal mice, peroxisomal VLACS was clearly lower in ALDP-deficient than in normal mice.

CONCLUSIONS: ALDP plays a role in the peroxisomal localization of VLACS, and VLACS does not function unless localized in the peroxisome.




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