PMID: 37504Jun 1, 1979Paper

Mitrochondrial NADH dehydrogenase in cystic fibrosis

Proceedings of the National Academy of Sciences of the United States of America
B L ShapiroL F Lam

Abstract

We have shown that skin fibroblast from patients with cystic fibrosis (CF) and from carriers for CF [heterozygotes (HZ)] consume more O2 than do their controls. When the mitochondrial electron transport inhibitor rotenone was added to the cells, the relative inhibition of O2 consumption was CF greater than HZ greater than controls (P less than 0.005 in both comparisons). Because rotenone specifically inhibits NADH dehydrogenase, [NADH: (acceptor) oxidoreductase, EC 1.6.99.3], which is the enzyme of energy-conserving site 1 of the mitochondrial electron transport system, activity and kinetics of this enzyme system were studied in fibroblast homogenates. NADH dehydrogenase activity was equal in cells from the three genotypes. At pH 8.0, affinity of the enzyme for its substrate was CF greater than HZ = controls; at pH 8.6, affinity was CF greater than HZ = controls (P less than 0.005 for the differences). pH optima for the genotypes were without exception 8.6 (CF), 8.3 (HZ), and 8.0 (control). HZ and control lines were distinguished unequivocally in a blind test on the basis of differences in pH optima. Purified mitochondrial preparations revealed pH optima identical to those found in whole cell homogenates. These data suggest tha...Continue Reading

References

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Citations

Jan 1, 1989·Life Sciences·B L Shapiro
Apr 27, 2011·Human Molecular Genetics·Mairead Kelly-AubertAleksander Edelman
Jan 1, 1986·Annals of the New York Academy of Sciences·R J Feigal, B L Shapiro
Sep 23, 2003·American Journal of Respiratory and Critical Care Medicine·Hiran C SelvaduraiPeter P Van Asperen
Jun 10, 2006·American Journal of Respiratory Cell and Molecular Biology·Leonard W VelsorBrian J Day
Sep 12, 2013·Redox Biology·Angel Gabriel Valdivieso, Tomás A Santa-Coloma
Dec 3, 2014·Neurobiology of Learning and Memory·Ritu JoshiSairam Krishnamurthy
Dec 19, 2013·Pharmacology, Biochemistry, and Behavior·Milind GeedSairam Krishnamurthy
Mar 27, 2007·Biochemical and Biophysical Research Communications·Angel G ValdiviesoTomás A Santa-Coloma
Feb 13, 2018·Oxidative Medicine and Cellular Longevity·Natalia El-MerhieSrikanth Karnati
Apr 21, 1986·FEBS Letters·M BattinoG Lenaz
Jun 19, 2019·American Journal of Physiology. Endocrinology and Metabolism·Nicola LaiCharles Hoppel
Nov 9, 2019·Journal of Clinical Medicine·Maria FaviaAnna Atlante
Oct 28, 2019·Naunyn-Schmiedeberg's Archives of Pharmacology·Akanksha Mishra, Sairam Krishnamurthy
Jan 10, 2020·Neuromolecular Medicine·Debapriya Garabadu, Nidhi Agrawal
Dec 1, 1982·Clinical Genetics·N R Sanguinetti-Briceno, D J Brock
Feb 9, 2017·Cellular and Molecular Neurobiology·Alok TripathiSairam Krishnamurthy
Jan 2, 2019·Cellular and Molecular Life Sciences : CMLS·Ángel G ValdiviesoTomás A Santa-Coloma
Mar 2, 2017·The Journal of Physiology·Paula Rodriguez-MiguelezRyan A Harris
Aug 28, 2020·Frontiers in Immunology·Giulio CabriniRoberto Gambari
Jul 29, 2020·Laboratory Investigation; a Journal of Technical Methods and Pathology·Keyan ZareiDavid A Stoltz
Oct 27, 2020·Frontiers in Pharmacology·Simone PatergnaniAlessandro Rimessi
Apr 13, 2021·The International Journal of Biochemistry & Cell Biology·Rocío GarcíaÁngel G Valdivieso
May 11, 2021·Purinergic Signalling·Edward H AbrahamKent Robinson

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