Mitochondrial intermembrane inclusion bodies: The common denominator between human mitochondrial myopathies and creatine depletion, due to impairment of cellular energetics

Intracellular compartmentation, structure and function of creatine kinase isoenzymes in tissues with high and fluctuating energy demands: the ‘phosphocreatine circuit’ for cellular energy homeostasis

1992 • 1,958 citations

DISEASES OF THE MITOCHONDRIAL DNA

1992 • 1,294 citations

A CASE OF SEVERE HYPERMETABOLISM OF NONTHYROID ORIGIN WITH A DEFECT IN THE MAINTENANCE OF MITOCHONDRIAL RESPIRATORY CONTROL: A CORRELATED CLINICAL, BIOCHEMICAL, AND MORPHOLOGICAL STUDY

1962 • 943 citations

Role of creatine phosphokinase in cellular function and metabolism

1978 • 298 citations

Structure of mitochondrial creatine kinase

1996 • 291 citations

Muscle-type MM creatine kinase is specifically bound to sarcoplasmic reticulum and can support Ca2+ uptake and regulate local ATP/ADP ratios.

1990 • 207 citations

Experimental ischemic myopathy

1974 • 177 citations

Native mitochondrial creatine kinase forms octameric structures. I. Isolation of two interconvertible mitochondrial creatine kinase forms, dimeric and octameric mitochondrial creatine kinase: characterization, localization, and structure-function relationships.

1988 • 167 citations

Phosphagen and intracellular pH changes during contraction of creatine-depleted rat muscle

1986 • 147 citations

Biochemical adaptation in the skeletal muscle of rats depleted of creatine with the substrate analogue β-guanidinopropionic acid

1985 • 133 citations

Differential effects of creatine depletion on the regulation of enzyme activities and on creatine-stimulated mitochondrial respiration in skeletal muscle, heart, and brain

1996 • 129 citations

Mitochondrial creatine kinase mediates contact formation between mitochondrial membranes.

1991 • 129 citations

Specificity of creatine kinase. New glycocyamines and glycocyamine analogs related to creatine

1971 • 128 citations

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

1993 • 102 citations

Creatine metabolism and the consequences of creatine depletion in muscle

1994 • 101 citations

Mitochondrial creatine kinase: a major constituent of pathological inclusions seen in mitochondrial myopathies.

1994 • 90 citations

Mitochondrial changes in ischemic skeletal muscle

1977 • 84 citations

Adult rat cardiomyocytes cultured in creatine-deficient medium display large mitochondria with paracrystalline inclusions, enriched for creatine kinase.

1991 • 81 citations

Actions of the creatine analogue β-guanidinopropionic acid on rat heart mitochondria

1994 • 47 citations

Two types of mitochondrial crystals in diseased human skeletal muscle fibers

1988 • 40 citations

Mitochondria in degenerating and regenerating skeletal muscle.

1969 • 39 citations

Creatine treatment in MELAS.

1994 • 36 citations

Crystalline mitochondrial inclusion bodies isolated from creatine depleted rat soleus muscle

1997 • 34 citations

Crystallization of Mitochondrial Creatine Kinase on Negatively Charged Lipid Layers

1994 • 31 citations

Effects of ?-guanidinopropionic acid-feeding on the patterns of myosin isoforms in rat fast-twitch muscle

1995 • 28 citations

Cryofixation of suspensions and tissues by propane-jet freezing and high-pressure freezing

1984 • 27 citations

Intramitochondrial inclusions caused by depletion of creatine in rat skeletal muscles.

1988 • 23 citations

Granular nuclear inclusion body disease: Fine structure of tibial muscle and sural nerve

1985 • 19 citations

Mitochondrial myopathy in rats fed with a diet containing beta-guanidine propionic acid, an inhibitor of creatine entry in muscle cells.

1988 • 16 citations