title: The Km of NADH dehydrogenase is decreased in mitochondria of cystic fibrosis cells.
abstract: The kinetic properties of the NADH dehydrogenase of the mitochondrial respiratory chain, assayed as NADH-dependent rotenone-sensitive cytochrome c reductase have been studied in mitochondria isolated from mononuclear white blood cells in patients affected by cystic fibrosis. Data reported here show that the apparent Km of the enzyme for NADH is significantly decreased in cystic fibrosis mitochondria. These findings are independent of the age or the clinical state of the disease and have also been obtained with mitochondria isolated from cultured skin fibroblasts. These observations support the notion that cystic fibrosis is possibly accompanied by alterations of intracellular membranes and these are evident also in circulating cells and cultured fibroblasts.
authors.affiliation: Centro Fibrosi Cistica, Ospedale di Borgo Trento, Italia.
authors.forename: M C
authors.initials: MC
authors.lastname: Dechecchi
authors.forename: E
authors.initials: E
authors.lastname: Girella
authors.forename: G
authors.initials: G
authors.lastname: Cabrini
authors.forename: G
authors.initials: G
authors.lastname: Berton
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000201:enzymology*; D003579:Cytochrome Reductases / Q000378:metabolism*; D006801:Humans; D007223:Infant; D007700:Kinetics; D007962:Leukocytes / Q000201:enzymology / Q000648:ultrastructure; D008928:Mitochondria / Q000201:enzymology*; D009245:NADH Dehydrogenase / Q000378:metabolism*; D012402:Rotenone / Q000494:pharmacology
chemical_list: D012402:Rotenone; D003579:Cytochrome Reductases; D009245:NADH Dehydrogenase