Article
Cerebral inflammation in X-linked adrenoleukodystrophy.
Archivum immunologiae et therapiae experimentalis - 1 Jan 1999
McGuinness M C, Smith K D
Abstract excerpt
X-linked adrenoleukodystrophy (X-ALD) is an inherited neurodegenerative disease that affects approximately 1 in 25 000 males. It is characterized by elevated levels of saturated very long chain fatty acids (VLCFA), i.e., >C22:0, particularly in ganglioside and cholesterol ester fractions of brain white matter and adrenal cortex. Failure of peroxisomal very long chain fatty acyl-CoA synthetase (VLCS) to activate...
Topics
- ATP Binding Cassette Transporter, Subfamily D, Member 1
- ATP-Binding Cassette Transporters
- Adrenoleukodystrophy
- Age of Onset
- Brain
- Brain Chemistry
- Child
- Cytokines
- Demyelinating Diseases
- Fatty Acids
- Genetic Heterogeneity
- Genetic Predisposition to Disease
- Genotype
- HLA Antigens
- Humans
- Major Histocompatibility Complex
- Male
- Membrane Proteins