adrenoleukodystrophy

Glyceryl Trioleate has been investigated for the treatment of Adrenoleukodystrophy.

L-Pipecolic acid (L-Homoproline) is a breakdown product of lysine, accumulates in body fluids of infants with generalized genetic peroxisomal disorders, such as Zellweger syndrome, neonatal adrenoleukodystrophy.

Tridecanedioic acid (Brassilic acid) is an unusual odd-numbered dicarboxylic acid that appears in the urines of children with neonatal adrenoleukodystrophy and Zellweger syndrome, as an additional marker of these peroxisomal disorders.

Nervonic acid (Selacholeic acid) is a long chain unsaturated fatty acid that is enriched in sphingomyelin. It consists of choline, sphingosine, phosphoric acid, and fatty acid. Nervonic acid may enhance the brain functions and prevent demyelination (Chemical Land21). Research shows that there is negative relationship between nervonic acid and obesity-related risk factors. Demyelination in adrenoleukodystrophy (ALD) is associated with an accumulation of very long chain saturated fatty acids stemming from a genetic defect in the peroxisomal beta oxidation system responsible for the chain shortening of these fatty acids. Sphingolipids from post mortem ALD brain have decreased levels of nervonic acid, 24:1(n-9), and increased levels of stearic acid, 18:0.

Hexacosanoic acid, a very long-chain fatty acid, is related to adrenoleukodystrophy (ALD), adrenomyeloneuropathy (AMN) and atherosclerosis.

Pentacosanoic acid, belongs to the class of organic compounds known as very long-chain fatty acids.