Evidence for a mitochondrial lesion in cystic fibrosis

Molecular Cell Biology

1986 • 5,808 citations

AN ELECTRON-TRANSPORT SYSTEM ASSOCIATED WITH THE OUTER MEMBRANE OF LIVER MITOCHONDRIA

1967 • 2,311 citations

Chloride impermeability in cystic fibrosis

1983 • 750 citations

Altered Regulation of Airway Epithelial Cell Chloride Channels in Cystic Fibrosis

1986 • 568 citations

Calcium Rises Abruptly and Briefly Throughout the Cell at the Onset of Anaphase

1986 • 541 citations

Chloride and potassium channels in cystic fibrosis airway epithelia

1986 • 392 citations

Perspectives in Cystic Fibrosis

1979 • 276 citations

Structure of NADH-Ubiquinone Reductase (Complex I)

1987 • 150 citations

Cystic Fibrosis: Horizons

1985 • 148 citations

INCREASED ENERGY EXPENDITURE IN YOUNG CHILDREN WITH CYSTIC FIBROSIS

1988 • 141 citations

Deficiency of the iron-sulfur clusters of mitochondrial reduced nicotinamide-adenine dinucleotide-ubiquinone oxidoreductase (complex I) in an infant with congenital lactic acidosis.

1984 • 138 citations

Mitochondrial calcium uptake and oxygen consumption in cystic fibrosis

1979 • 125 citations

Increased resting energy expenditure in cystic fibrosis

1988 • 112 citations

Oxygen Consumption and Ouabain Binding Sites in Cystic Fibrosis Nasal Epithelium

1986 • 104 citations

Microscopic Nephrocalcinosis in Cystic Fibrosis

1988 • 87 citations

Ruthenium red as a probe in assessing the potential of mitochondria to control intracellular calcium in liver

1977 • 58 citations

Molecular cell biology

1986 • 47 citations

Altered Intracellular Calcium in Fibroblasts from Patients with Cystic Fibrosis and Heterozygotes

1979 • 43 citations

Isolated Parotid Acinar Cells from Patients with Cystic Fibrosis. Morphology and Composition

1981 • 41 citations

Calcium Metabolism and Cystic Fibrosis: Mitochondrial Abnormalities Suggest a Modification of the Mitochondrial Membrane

1984 • 40 citations

Brillouin scattering study of equation of state of single component liquids

1988 • 40 citations

Identification of a defective cAMP-stimulated Cl- channel in cystic fibrosis fibroblasts.

1987 • 36 citations

Doubling time α-aminoisobutyrate transport and calcium exchange in cultured fibroblasts from cystic fibrosis and control subjects

1978 • 33 citations

Calcium and Age in Fibroblasts from Control Subjects and Patients with Cystic Fibrosis

1982 • 31 citations

Mitochondrial NADH dehydrogenase in cystic fibrosis: enzyme kinetics in cultured fibroblasts.

1982 • 30 citations

Kinetic analysis of chloride efflux from normal and cystic fibrosis fibroblasts

1986 • 28 citations

Cytosolic free calcium concentration and intracellular calcium distribution in lymphocytes from cystic fibrosis patients

1984 • 26 citations

Cystic Fibrosis: Lysosomal and Mitochondrial Enzyme Activities of Lymphoid Cell Lines

1972 • 26 citations

The calcium abnormality in cystic fibrosis mitochondria: Relative role of respiration and ATP hydrolysis

1982 • 25 citations

Is cytoplasmic Ca2+ in lymphocytes elevated in cystic fibrosis

1984 • 24 citations

The K(m) of NADH Dehydrogenase Is Decreased in Mitochondria of Cystic Fibrosis Cells

1988 • 23 citations

The Search for the Cystic Fibrosis Gene

1986 • 22 citations

Cell Calcium Levels of Normal and Cystic Fibrosis Nasal Epithelium

1988 • 20 citations

Kinetic alterations of cytochrome‐c oxidase in cystic fibrosis

1986 • 19 citations

Cystic fibrosis serum promotes [45Ca] uptake by normal human leukocytes

1978 • 15 citations

Intracellular calcium in cystic fibrosis heterozygotes

1987 • 13 citations

Increased Monocyte Chemiluminescence in Cystic Fibrosis Patients and in their Parents

1986 • 13 citations

Cystic Fibrosis?A Lethal Exocrinopathy with Altered Mitochondrial Calcium Metabolism

1986 • 9 citations

Cystic Fibrosis?A Lethal Exocrinopathy with Altered Mitochondrial Calcium Metabolism

1986 • 9 citations

NADH dehydrogenase in cystic fibrosis

1982 • 3 citations

Hormonal Regulation of Intramitochondrial Ca2+ and Oxidative Metabolism in Mammalian Tissues

1988 • 2 citations

Competitive Inhibition of Human Mitochondrial NADH Dehydrogenase by Cibacron Blue F3GA

1984 • 2 citations