Impairment of mitochondrial function in skeletal muscle of patients with amyotrophic lateral sclerosis

Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis

1993 • 6,762 citations

Methoden der enzymatischen Analyse

1962 • 3,983 citations

Motor Neuron Degeneration in Mice that Express a Human Cu,Zn Superoxide Dismutase Mutation

1994 • 3,959 citations

Aging, energy, and oxidative stress in neurodegenerative diseases

1995 • 1,453 citations

An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria

1995 • 1,424 citations

The control of flux.

1973 • 1,381 citations

DISEASES OF THE MITOCHONDRIAL DNA

1992 • 1,294 citations

Role of uncoupled and non-coupled oxidations in maintenance of safely low levels of oxygen and its one-electron reductants

1996 • 699 citations

Superoxide Dismutase Activity, Oxidative Damage, and Mitochondrial Energy Metabolism in Familial and Sporadic Amyotrophic Lateral Sclerosis

1993 • 627 citations

Evidence that mitochondrial respiration is a source of potentially toxic oxygen free radicals in intact rabbit hearts subjected to ischemia and reflow.

1993 • 518 citations

Action of Saponin on Biological Cell Membranes

1962 • 506 citations

Defective axonal transport in a transgenic mouse model of amyotrophic lateral sclerosis

1995 • 464 citations

Mitochondrial respiratory parameters in cardiac tissue: A novel method of assessment by using saponin-skinned fibers

1987 • 342 citations

Age-Dependent Penetrance of Disease in a Transgenic Mouse Model of Familial Amyotrophic Lateral Sclerosis

1995 • 317 citations

Action of Saponin on Biological Cell Membranes

1962 • 250 citations

A Linear Steady-State Treatment of Enzymatic Chains. Critique of the Crossover Theorem and a General Procedure to Identify Interaction Sites with an Effector

1974 • 239 citations

The Golgi apparatus of spinal cord motor neurons in transgenic mice expressing mutant Cu,Zn superoxide dismutase becomes fragmented in early, preclinical stages of the disease.

1996 • 224 citations

Intracellular Oxidation-Reduction State Measured in Situ by a Multichannel Fiber-Optic Surface Fluorometer

1982 • 222 citations

Ultrastructural study of synapses in the anterior horn neurons of patients with amyotrophic lateral sclerosis

1996 • 170 citations

Structural and functional properties of adult rat heart myocytes lysed with digitonin.

1985 • 108 citations

Functional characterization of mitochondrial oxidative phosphorylation in saponin-skinned human muscle fibers

1993 • 102 citations

Application of inhibitor titrations for the detection of oxidative phosphorylation defects in saponin-skinned muscle fibers of patients with mitochondrial diseases

1997 • 61 citations

Measurement of Fluorescence Changes of NAD(P)H and of Fluorescent Flavoproteins in Saponin-Skinned Human Skeletal Muscle Fibers

1994 • 27 citations

Laser-excited fluorescence studies of mitochondria) function in saponin-skinned skeletal muscle fibers of patients with chronic progressive external ophthalmoplegia

1995 • 14 citations