ABSTRACT
A congenital disorder syndrome defined by literature as the Alperts'-Crouzon has been described for a six-year-old boy. Introduction of this case seems to be justified noting the rarity of its occurrence.
Subject(s)
Acrocephalosyndactylia/diagnosis , Craniofacial Dysostosis/diagnosis , Child , Humans , Male , SyndromeSubject(s)
Calcium/metabolism , Kidney Cortex/metabolism , Linolenic Acids/pharmacology , Mitochondria/metabolism , Animals , Biological Transport/drug effects , Carbonyl Cyanide m-Chlorophenyl Hydrazone/pharmacology , Cations, Monovalent , Female , Kidney Cortex/drug effects , Kinetics , Mitochondria/drug effects , Mitochondria, Liver/metabolism , Organ Specificity , Rats , Serum Albumin, Bovine/pharmacology , Sodium/pharmacologyABSTRACT
The effects of fatty acids and monovalent cations on the Ca2+ efflux from isolated liver and kidney mitochondria were investigated by means of electrode techniques. It was shown that unsaturated fatty acids and saturated fatty acids of medium chain length (C12 and C14) induced a Ca2+ efflux from mitochondria which was not inhibited by ruthenium red, but was specifically inhibited by Na+ and Li+. The Ca2+-releasing activity of unsaturated fatty acids did not correlate with their uncoupling activity. In kidney mitochondria a spontaneous, temperature-dependent Ca2+ efflux was observed which was inhibited either by albumin or by Na+. It is suggested that the net Ca2+ accumulation by mitochondria depends on the operation of independent pump and leak pathways. The pump is driven by the membrane potential and can be inhibited by ruthenium red, the leak depends on the presence of unsaturated fatty acids and is inhibited by Na+ and Li+. It is suggested that the unsaturated fatty acids produced by mitochondrial phospholipase A2 can be essential in the regulation of the Ca2+ retention in and the Ca2+ release from the mitochondria.
