Disease relevance of Cerebronic acid

• On the other hand, alpha-oxidation of cerebronic acid was found to be normal in cell lines from X-linked adrenoleukodystrophy, adult Refsum disease, and rhizomelic chondrodysplasia punctata [1].
• Studies on cultured fibroblasts from normal subjects and patients with peroxisomal disorders indicated an impairment of alpha-oxidation of cerebronic acid in cell lines that lack peroxisomes [e.g., Zellweger syndrome (ZS)] [1].

High impact information on Cerebronic acid

• The preparations of brains from jimpy and myelin synthesis deficiency (msd) mice were found to synthesize cerebronic acid at less than 10 percent of their control rates, and those from quaking and dilute-lethal approximately 30 and 50 percent, respectively [2].
• Alpha Hydroxylation of lignoceric acid (n-tetracosanoic acid) to cerebronic acid (2-hydroxylignoceric acid) by postnuclear preparations of brains from developing rat, mouse, and several neurological mouse mutants was studied [2].
• These results suggest a close association of the synthesis of cerebronic acid with the synthesis of the characteristic myelin lipid that is cerebroside (N-acyl sphingosine beta-D-galactoside) [2].
• When the microsomes of FA2H-transfected COS7 cells were incubated with the electron transfer system and deuterated tetracosanoic acid, deuterated 2-hydroxy tetracosanoic acid was formed in a time- and protein-dependent manner [3].
• Identification of the pathway of alpha-oxidation of cerebronic acid in peroxisomes [1].

Biological context of Cerebronic acid

• The apparent low rate of in vitro hydroxylation by brains of the mutant mice appeared to be due to decreased synthesis rather than increased oxidation of cerebronic acid [2].

Anatomical context of Cerebronic acid

• Intraorganellar localization studies indicated that the enzyme system for the alpha-oxidation of cerebronic acid was associated with the peroxisomal limiting membranes [1].

Analytical, diagnostic and therapeutic context of Cerebronic acid

• The description, which is tested in a combined microscopy and rheology study of the collapse of a single-phase Langmuir monolayer (LM) of 2-hydroxy-tetracosanoic acid (2-OH TCA), provides a connection between the in-plane rheology of LMs and reversible folding [4].

References