Probing substrate-induced conformational alterations in adrenoleukodystrophy protein by proteolysis

• Tipo: Artigo em Revista Científica Internacional
• Data: 2005

Autores

guimaraes, cp (Autor) Outra; sa-miranda, c (Autor) Outra; azevedo, je (Autor) ICBAS

Revista

Título: Journal of Human Genetics

Vol. 50

Páginas: 99-105

ISSN: 1434-5161

Editora: Springer Nature

Indexação

ISI Web of Knowledge - 10 Citações

Scopus - 10 Citações

Outras Informações

ID Authenticus: P-000-4WY

DOI: 10.1007/s10038-004-0226-4

Abstract (EN): The adrenoleukodystrophy protein (ALDP) is a half-ABC (ATP-binding cassette) transporter localized in the peroxisomal membrane. Dysfunction of this protein is the cause of the human genetic disorder X-linked adrenoleukodystrophy (X-ALD), which is characterized by accumulation of saturated, very-long-chain fatty acids (VLCFAs). This observation suggests that ALDP is involved in the metabolism of these compounds. Whether ALDP transports VLCFAs or their derivatives across the peroxisomal membrane or some cofactors essential for the efficient peroxisomal b-oxidation of these fatty acids is still unknown. In this work, we used a protease-based approach to search for substrate-induced conformational alterations on ALDP. Our results suggest that ALDP is directly involved in the transport of long- and very-long-chain acyl-CoAs across the peroxisomal membrane.

Idioma: Inglês

Tipo (Avaliação Docente): Científica

Nº de páginas: 7