title: Identification of retinoyl complexes as the autofluorescent component of the neuronal storage material in Batten disease.
abstract: 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.
mesh_terms: D001921:Brain / Q000378:metabolism* / Q000473:pathology; D002648:Child; D006801:Humans; D008064:Lipidoses / Q000378:metabolism* / Q000473:pathology; D008566:Membranes / Q000378:metabolism; D008854:Microscopy, Electron; D009419:Nerve Tissue Proteins / Q000378:metabolism; D010455:Peptides / Q000378:metabolism; D013050:Spectrometry, Fluorescence; D014212:Tretinoin / Q000031:analogs & derivatives* / Q000378:metabolism; D014801:Vitamin A / Q000031:analogs & derivatives*
chemical_list: D009419:Nerve Tissue Proteins; D010455:Peptides; D014801:Vitamin A; D014212:Tretinoin