On the neurochemistry of an adult form of ceroid lipofuscinosis (Kuf's disease).
Little progress has been made in procedures for diagnosis of this baffling group of diseases known as ceroid lipofuscinoses (CL), which exhibit infantile, juvenile, and adult forms. Although these have been shown for the most part to be genetic in origin, the enzymatic or other defects leading to accumulation of lipopigments in tertiary lysosomes of neurons or astrocytes re unknown. Among the more commonly discussed theories is the role of peroxy radicals or their products formed by lipoperoxidation of polyunsaturated fatty acids. Although peroxy radicals are damaging to tissues and biological processes, no differences were observed in levels of leucocyte peroxidase (metabolizing H2O2) for Kuf's patients in comparison to age-matched controls. Also, no differences were observed in levels of blood factors that affect the rates of lipoperoxidatin by rat or human brain homogenates in vitro (measured by formation of MDA or other TBA positive materials).
D000328:Adult; D000818:Animals; D001923:Brain Chemistry; D002566:Ceroid / Q000378:metabolism*; D005227:Fatty Acids / Q000032:analysis; D006801:Humans; D007223:Infant; D007962:Leukocytes / Q000201:enzymology; D008054:Lipid Peroxides / Q000378:metabolism; D008062:Lipofuscin / Q000378:metabolism*; D010544:Peroxidases / Q000378:metabolism; D010859:Pigmentation Disorders / Q000378:metabolism*; D010860:Pigments, Biological / Q000378:metabolism*; D051381:Rats; D013577:Syndrome
D002566:Ceroid; D005227:Fatty Acids; D008054:Lipid Peroxides; D008062:Lipofuscin; D010860:Pigments, Biological; D010544:Peroxidases