Nutritional cofactor treatment in mitochondrial disorders (Q34218646)

31 July 2017

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title

Nutritional cofactor treatment in mitochondrial disorders (English)

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31 July 2017

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Barbara Marriage

1

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PubMed ID

12891154

31 July 2017

M Thomas Clandinin

series ordinal

2

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PubMed ID

12891154

31 July 2017

D Moira Glerum

series ordinal

3

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31 July 2017

1 August 2003

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Journal of the American Dietetic Association

31 July 2017

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31 July 2017

103

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31 July 2017

8

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31 July 2017

1029-1038

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Clinical spectrum and diagnosis of mitochondrial disorders

31 July 2017

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Incidence of inborn errors of metabolism in British Columbia, 1969-1996.

7 January 2021

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Biochemical, physiological and medical aspects of ubiquinone function

7 January 2021

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Fluidizing effect of endogenous ubiquinone in bovine heart mitochondrial membranes

7 January 2021

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Marked reduction in CSF lactate and pyruvate levels after CoQ therapy in a patient with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS)

7 January 2021

1 reference

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Clinical and biochemical correlations in mitochondrial myopathies treated with coenzyme Q10

7 January 2021

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Long-term coenzyme Q10 therapy for a mitochondrial encephalomyopathy with cytochrome c oxidase deficiency: a 31P NMR study

7 January 2021

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Clinical improvement after administration of coenzyme Q10 in a patient with mitochondrial encephalomyopathy

7 January 2021

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Improvement of abnormal pyruvate metabolism and cardiac conduction defect with coenzyme Q10 in Kearns-Sayre syndrome

7 January 2021

1 reference

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Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes with recurrent abdominal symptoms and coenzyme Q10 administration

7 January 2021

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Mitochondrial encephalomyopathy (MELAS): pathological study and successful therapy with coenzyme Q10 and idebenone

7 January 2021

1 reference

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Effect of coenzyme Q10 in patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS): evaluation by noninvasive tissue oximetry

7 January 2021

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31P NMR spectroscopy and ergometer exercise test as evidence for muscle oxidative performance improvement with coenzyme Q in mitochondrial myopathies

7 January 2021

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Two successful double-blind trials with coenzyme Q10 (vitamin Q10) on muscular dystrophies and neurogenic atrophies

7 January 2021

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Coenzyme Q10 treatment in mitochondrial encephalomyopathies. Short-term double-blind, crossover study

7 January 2021

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The effect of coenzyme Q10 on blood glucose and insulin requirement in patients with insulin dependent diabetes mellitus

7 January 2021

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Phosphorus magnetic resonance spectroscopy in the evaluation of mitochondrial myopathies: results of a 6-month therapy study with coenzyme Q.

7 January 2021

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Ubidecarenone in the treatment of mitochondrial myopathies: a multi-center double-blind trial.

7 January 2021

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Metabolic changes in patients with mitochondrial myopathies and effects of coenzyme Q10 therapy

7 January 2021

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The effects of coenzyme Q10 treatment on maternally inherited diabetes mellitus and deafness, and mitochondrial DNA 3243 (A to G) mutation.

7 January 2021

1 reference

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Familial cerebellar ataxia with muscle coenzyme Q10 deficiency

7 January 2021

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Quinone-responsive multiple respiratory-chain dysfunction due to widespread coenzyme Q10 deficiency

7 January 2021

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Mitochondrial encephalomyopathy with coenzyme Q10 deficiency

7 January 2021

1 reference

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Bioavailability of four oral coenzyme Q10 formulations in healthy volunteers

7 January 2021

1 reference

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A one year bioavailability study of coenzyme Q10 with 3 months withdrawal period

7 January 2021

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Effect of Oral Coenzyme Q10 Supplementation on the Oxidation Resistance of Human VLDL+LDL Fraction: Absorption and Antioxidative Properties of Oil and Granule-Based Preparations

7 January 2021

1 reference

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Treatment of mitochondrial cytopathies

7 January 2021

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Therapy of mitochondrial disorders

7 January 2021

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31P NMR study of improvement in oxidative phosphorylation by vitamins K3 and C in a patient with a defect in electron transport at complex III in skeletal muscle.

7 January 2021

1 reference

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alpha-Tocopherol/lipid ratio in blood is decreased in patients with Leber's hereditary optic neuropathy and asymptomatic carriers of the 11778 mtDNA mutation

7 January 2021

1 reference

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Prevention of mitochondrial oxidative damage using targeted antioxidants

7 January 2021

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Comparison of the effect of α-lipoic acid and α-tocopherol supplementation on measures of oxidative stress

7 January 2021

1 reference

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Lipoic (thioctic) acid increases brain energy availability and skeletal muscle performance as shown by in vivo 31P-MRS in a patient with mitochondrial cytopathy

7 January 2021

1 reference

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NADH-CoQ reductase deficient myopathy: successful treatment with riboflavin

7 January 2021

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Treatment of complex I deficiency with riboflavin

7 January 2021

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Mitochondrial myopathy with tRNA(Leu(UUR)) mutation and complex I deficiency responsive to riboflavin

7 January 2021

1 reference

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MELAS syndrome with mitochondrial tRNA(Leu)(UUR) mutation: correlation of clinical state, nerve conduction, and muscle 31P magnetic resonance spectroscopy during treatment with nicotinamide and riboflavin

7 January 2021

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Riboflavin-responsive complex I deficiency

7 January 2021

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Successful treatment of pure myopathy, associated with complex I deficiency, with riboflavin and carnitine

7 January 2021

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A mitochondrial myopathy in an infant with lactic acidosis.

7 January 2021

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7 January 2021

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Biogenesis of flavoprotein and cytochrome components in hepatic mitochondria from riboflavin-deficient rats

7 January 2021

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Effect of various agents on adenosine triphosphate synthesis in mitochondrial complex I deficiency

7 January 2021

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Molecular analysis of abnormal pyruvate dehydrogenase in a patient with thiamine-responsive congenital lactic acidemia

7 January 2021

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Correction of increased plasma pyruvate and plasma lactate levels using large doses of thiamine in patients with Kearns-Sayre syndrome

7 January 2021

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Mitochondrial myopathy and familial thiamine deficiency

7 January 2021

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Increase of blood NAD+ and attenuation of lactacidemia during nicotinamide treatment of a patient with the MELAS syndrome

7 January 2021

1 reference

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Treatment of mitochondrial myopathy due to complex III deficiency with vitamins K3 and C: A 31P-NMR follow-up study

7 January 2021

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Early-onset cerebellar ataxia, myoclonus and hypogonadism in a case of mitochondrial complex III deficiency treated with vitamins K3 and C

7 January 2021

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Vitamin K-dependent carboxylase

7 January 2021

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Brain 31P-magnetic resonance spectroscopy in mitochondrial cytopathies

7 January 2021

1 reference

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Assessment of mitochondrial function and control in normal and diseased states

7 January 2021

1 reference

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Elevation of creatine in resting and exercised muscle of normal subjects by creatine supplementation

7 January 2021

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A randomized, controlled trial of creatine monohydrate in patients with mitochondrial cytopathies

7 January 2021

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Clinical and biochemical aspects of carnitine deficiency and insufficiency: transport defects and inborn errors of beta-oxidation

7 January 2021

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CPEO and carnitine deficiency overlapping in MELAS syndrome.

7 January 2021

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Skin fibroblast carnitine uptake in secondary carnitine deficiency disorders

7 January 2021

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Plasma carnitine insufficiency and effectiveness of L-carnitine therapy in patients with mitochondrial myopathy

7 January 2021

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Abnormal carnitine distribution in the muscles of patients with idiopathic inflammatory myopathy

7 January 2021

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The treatment of mitochondrial myopathies and encephalomyopathies

7 January 2021

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Coenzyme Q10 with multiple vitamins is generally ineffective in treatment of mitochondrial disease

7 January 2021

1 reference

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10.1016/S0002-8223(03)00476-0

7 January 2021

Identifiers

DOI

1 reference

31 July 2017

1 reference

PubMed ID

12891154