Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy.

Abstract excerpt

BACKGROUND: X-ALD is a white matter (WM) disease caused by mutations in the ABCD1 gene encoding the transporter of very-long-chain fatty acids (VLCFAs) into peroxisomes. Strikingly, the same ABCD1 mutation causes either devastating brain inflammatory demyelination during childhood or, more often, progressive spinal cord axonopathy starting in middle-aged adults. The accumulation of undegraded VLCFA in glial cell...

Topics

• Adrenoleukodystrophy
• Animals
• Humans
• ATP Binding Cassette Transporter, Subfamily D, Member 1
• Mice
• Disease Models, Animal
• Fatty Acids