tretinoin and Lipidoses

Cytosomes filled with intensely fluorescent material in the form of curvilinear bodies were isolated by density gradient centrifugation followed by pronase digestion from the cerebral cortex of a child who had died at age 7 from the late infantile form of Batten disease. Forty-three percent of the dry weight of the storage material was extracted by a mixture of chloroform and methanol, leaving a waterinsoluble amorphous fluorescent residue. Infrared spectroscopy, proton magnetic resonance spectrscopy, and mass spectrometry of this residue strongly suggested the presence of retinoyl polyenes linked to a small peptide. Base hydrolysis and methanolysis yielded retinoic acid and methyl retinoate, respectively. Ozonolysis yielded a product derived from the substituted cyclohexenyl ring of vitamin A. The results indicate that the fluorescent component of the neuronal storage material is a retinoyl complex and is not derived from peroxidized polyunsatured fatty acids as previously thought.

Topics: Brain; Child; Humans; Lipidoses; Membranes; Microscopy, Electron; Nerve Tissue Proteins; Peptides; Spectrometry, Fluorescence; Tretinoin; Vitamin A

Topics: Cholesterol; Humans; Lipidoses; Neurons; Syndrome; Tretinoin; Vitamin A