Mitochondrial dysfunction and oxidative damage cooperatively fuel axonal degeneration in X-linked adrenoleukodystrophy
Abstract X-linked adrenoleukodystrophy (X-ALD) is the most frequent inherited monogenic
demyelinating disease (minimal incidence 1: 17,000). It is often lethal and currently lacks a
satisfactory therapy. The disease is caused by loss of function of the ABCD1 gene, a
peroxisomal ATP-binding cassette transporter, resulting in the accumulation of VLCFA (very
long-chain fatty acids) in organs and plasma. Understanding of the aetiopathogenesis is a
prerequisite for the development of novel therapeutic strategies. Functional genomics …
demyelinating disease (minimal incidence 1: 17,000). It is often lethal and currently lacks a
satisfactory therapy. The disease is caused by loss of function of the ABCD1 gene, a
peroxisomal ATP-binding cassette transporter, resulting in the accumulation of VLCFA (very
long-chain fatty acids) in organs and plasma. Understanding of the aetiopathogenesis is a
prerequisite for the development of novel therapeutic strategies. Functional genomics …
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