The mitochondrial myopathy encephalopathy, lactic acidosis with stroke-like episodes (MELAS) syndrome: a review of treatment options (Q36492305)

14 August 2017

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title

The mitochondrial myopathy encephalopathy, lactic acidosis with stroke-like episodes (MELAS) syndrome: a review of treatment options (English)

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14 August 2017

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Fernando Scaglia

1

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PubMed ID

16734497

14 August 2017

Jennifer L Northrop

series ordinal

2

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14 August 2017

1 January 2006

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14 August 2017

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14 August 2017

20

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14 August 2017

6

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14 August 2017

443-464

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https://scigraph.springernature.com/pub.10.2165/00023210-200620060-00002

14 August 2017

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cites work

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Cardiac involvement in mitochondrial diseases. A study on 17 patients with documented mitochondrial DNA defects

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21 January 2018

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A missense mutation in the mitochondrial ND5 gene associated with a Leigh-MELAS overlap syndrome.

21 January 2018

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Near-infrared spectroscopy in the diagnosis of mitochondrial disorders

21 January 2018

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Defects in mitochondrial protein synthesis and respiratory chain activity segregate with the tRNA(Leu(UUR)) mutation associated with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes

21 January 2018

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https://api.crossref.org/works/10.2165%2F00023210-200620060-00002

Neuronal hyperexcitability in stroke-like episodes of MELAS syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.2165%2F00023210-200620060-00002

Slowly progressive spread of the stroke-like lesions in MELAS

21 January 2018

1 reference

https://api.crossref.org/works/10.2165%2F00023210-200620060-00002

L-arginine improves the symptoms of strokelike episodes in MELAS.

21 January 2018

1 reference

https://api.crossref.org/works/10.2165%2F00023210-200620060-00002

Arginine metabolism: nitric oxide and beyond

21 January 2018

1 reference

https://api.crossref.org/works/10.2165%2F00023210-200620060-00002

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

21 January 2018

1 reference

https://api.crossref.org/works/10.2165%2F00023210-200620060-00002

Ubiquinone and nicotinamide treatment of patients with the 3243A-->G mtDNA mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.2165%2F00023210-200620060-00002

Antioxidative effect of ubiquinones on mitochondrial membranes

21 January 2018

1 reference

https://api.crossref.org/works/10.2165%2F00023210-200620060-00002

Disposition of idebenone (CV-2619), a new cerebral metabolism improving agent, in rats and dogs.

21 January 2018

1 reference

https://api.crossref.org/works/10.2165%2F00023210-200620060-00002

Idebenone improves cerebral mitochondrial oxidative metabolism in a patient with MELAS.

21 January 2018

1 reference

https://api.crossref.org/works/10.2165%2F00023210-200620060-00002

Treatment of experimental NADH ubiquinone reductase deficiency with menadione

21 January 2018

1 reference

https://api.crossref.org/works/10.2165%2F00023210-200620060-00002

Familial poliodystrophy, mitochondrial myopathy, and lactate acidemia.

21 January 2018

1 reference

https://api.crossref.org/works/10.2165%2F00023210-200620060-00002

Reversible alexia, mitochondrial myopathy, and lactic acidemia

21 January 2018

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https://api.crossref.org/works/10.2165%2F00023210-200620060-00002

Mitochondrial myopathy with cardiomyopathy, lactic acidosis and response to prednisone and thiamine

21 January 2018

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https://api.crossref.org/works/10.2165%2F00023210-200620060-00002

MELAS syndrome: Characteristic migrainous and epileptic features and maternal transmission

21 January 2018

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https://api.crossref.org/works/10.2165%2F00023210-200620060-00002

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

21 January 2018

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https://api.crossref.org/works/10.2165%2F00023210-200620060-00002

Some new aspects of creatine kinase (CK): compartmentation, structure, function and regulation for cellular and mitochondrial bioenergetics and physiology.

21 January 2018

1 reference

https://api.crossref.org/works/10.2165%2F00023210-200620060-00002

Effects of L-arginine on the acute phase of strokes in three patients with MELAS.

21 January 2018

1 reference

https://api.crossref.org/works/10.2165%2F00023210-200620060-00002

Phenotypes and mitochondrial DNA substitutions in families with A3243G mutation

21 January 2018

1 reference

12 December 2020

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

1 reference

12 December 2020

Strongly succinate dehydrogenase-reactive blood vessels in muscles from patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes

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12 December 2020

A MERRF/MELAS overlap syndrome associated with a new point mutation in the mitochondrial DNA tRNA(Lys) gene

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12 December 2020

The cerebral metabolism of glucose and oxygen measured with positron tomography in patients with mitochondrial diseases

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12 December 2020

Creatine treatment in MELAS

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12 December 2020

Diazepam inhibits cell respiration and induces fragmentation of mitochondrial reticulum.

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12 December 2020

Maternally inherited diabetes and deafness is a distinct subtype of diabetes and associates with a single point mutation in the mitochondrial tRNA(Leu(UUR)) gene.

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12 December 2020

Point mutation in platelet mitochondrial tRNA(Leu(UUR)) in patient with cluster headache

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12 December 2020

Dysfunction of the hypothalamic-pituitary system in mitochondrial encephalomyopathies.

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12 December 2020

Dichloroacetate treatment for mitochondrial cytopathy: long-term effects in MELAS.

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12 December 2020

Dichloroacetate inhibits glycolysis and augments insulin-stimulated glycogen synthesis in rat muscle

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12 December 2020

Clinical pharmacology and toxicology of dichloroacetate

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12 December 2020

Direct measurement of high-energy phosphate compounds in patients with neuromuscular disease

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12 December 2020

Effects of methylprednisolone and the combination of alpha-tocopherol and selenium on arachidonic acid metabolism and lipid peroxidation in traumatized spinal cord tissue

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12 December 2020

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

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12 December 2020

Beneficial effect of L-arginine for stroke-like episode in MELAS.

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12 December 2020

A case of mitochondrial cytopathy with a typical point mutation for MELAS, presenting with severe focal-segmental glomerulosclerosis as main clinical manifestation.

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12 December 2020

Regulation of the Human Pyruvate Dehydrogenase Complex

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12 December 2020

L-arginine-induced hypotension

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12 December 2020

Potential for creatine and other therapies targeting cellular energy dysfunction in neurological disorders

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12 December 2020

Varying loads of the mitochondrial DNA A3243G mutation in different tissues: implications for diagnosis

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12 December 2020

Quantitative proton magnetic resonance spectroscopy of cerebral metabolic disturbances in patients with MELAS

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12 December 2020

Possible biochemical mechanism of the toxic effects of barbiturates

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12 December 2020

Valproate-induced hepatic failure in a case of cytochrome c oxidase deficiency

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12 December 2020

Calcium Dependence of Toxic Cell Death: A Final Common Pathway

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12 December 2020

Vasogenic edema on MELAS: a serial study with diffusion-weighted MR imaging

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12 December 2020

Abnormal calcium homeostasis and mitochondrial polarization in a human encephalomyopathy

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12 December 2020

Heterogeneous presentation in A3243G mutation in the mitochondrial tRNA(Leu(UUR)) gene

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12 December 2020

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)

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12 December 2020

Concomitant administration of sodium dichloroacetate and vitamin B1 for lactic acidemia in children with MELAS syndrome

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12 December 2020

Dynamics of nitric oxide in the active site of reduced cytochrome c oxidase aa3

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12 December 2020

Mitochondrial DNA mutations and pathogenesis

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12 December 2020

Humanin improves impaired metabolic activity and prolongs survival of serum-deprived human lymphocytes

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12 December 2020

Metabolic interventions against complex I deficiency in MELAS syndrome

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12 December 2020

Improvement of insulin-stimulated glucose-disposal in type 2 diabetes after repeated parenteral administration of thioctic acid.

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12 December 2020

Magnetic resonance spectroscopy: use in monitoring MELAS treatment.

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12 December 2020

Effect of humanin on decreased ATP levels of human lymphocytes harboring A3243G mutant mitochondrial DNA.

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12 December 2020

Biochemical and genetic studies in a family with mitochondrial myopathy

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12 December 2020

Increased activities of antioxidant enzymes and decreased ATP concentration in cultured myoblasts with the 3243A-->G mutation in mitochondrial DNA

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12 December 2020

Bioenergetics of mitochondrial diseases associated with mtDNA mutations

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12 December 2020

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

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12 December 2020

Deficiency of subunits of Complex I and mitochondrial encephalomyopathy.

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12 December 2020

Nerve conduction changes in patients with mitochondrial diseases treated with dichloroacetate

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12 December 2020

Familial recurrence of atypical symptoms in an extended pedigree with the syndrome of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS)

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12 December 2020

Mitochondrial respiratory chain complex I deficiency with clinical and biochemical features of long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency

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12 December 2020

The A to G transition at nt 3243 of the mitochondrial tRNALeu(UUR) may cause an MERRF syndrome

1 reference

12 December 2020

Epidemiology of A3243G, the mutation for mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes: prevalence of the mutation in an adult population.

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12 December 2020

Treatment of mitochondrial cytopathies

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12 December 2020

Mitochondrial DNA and RNA processing in MELAS.

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12 December 2020

A rescue factor abolishing neuronal cell death by a wide spectrum of familial Alzheimer's disease genes and Aβ

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12 December 2020

Decrease of 3243 A-->G mtDNA mutation from blood in MELAS syndrome: a longitudinal study

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12 December 2020

MELAS syndrome: peripheral neuropathy and cytochrome C-oxidase deficiency: a case report and review of the literature.

1 reference

12 December 2020

Leigh disease with deficiency of lipoamide dehydrogenase: treatment failure with dichloroacetate

1 reference

12 December 2020

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

1 reference

12 December 2020

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

1 reference

12 December 2020

Effects of sodium valproate on mitochondrial membranes: electron paramagnetic resonance and transmembrane protein movement studies

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12 December 2020

Diseases of the mitochondrial DNA.

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12 December 2020

The epidemiology of pathogenic mitochondrial DNA mutations

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12 December 2020

Mitochondrial angiopathy in cerebral blood vessels of mitochondrial encephalomyopathy

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12 December 2020

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

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12 December 2020

The L-arginine-nitric oxide pathway

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12 December 2020

Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS): current concepts

1 reference

12 December 2020

Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) triggered by valproate therapy

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12 December 2020

Cytochrome c/cytochrome c oxidase interaction. Direct structural evidence for conformational changes during enzyme turnover

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12 December 2020

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

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12 December 2020

MELAS: clinical features, biochemistry, and molecular genetics

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12 December 2020

Effects of oral creatine supplementation in a patient with MELAS phenotype and associated nephropathy

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12 December 2020

Increased mitochondrial processing intermediates associated with three tRNA(Leu(UUR)) gene mutations.

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12 December 2020

Decreased ATP synthesis is phenotypically expressed during increased energy demand in fibroblasts containing mitochondrial tRNA mutations

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12 December 2020

Mitochondrial diseases

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12 December 2020

Treatment of mitochondrial encephalomyopathy with a combination of cytochrome C and vitamins B1 and B2.

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12 December 2020

Long-term treatment with idebenone and riboflavin in a patient with MELAS.

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12 December 2020

Use of near-infrared spectroscopy to monitor tissue oxygenation.

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12 December 2020

Hypocitrullinemia in patients with MELAS: an insight into the "MELAS paradox".

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12 December 2020

Mitochondrial encephalomyopathies: what next?

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12 December 2020

(R)-alpha-lipoic acid-supplemented old rats have improved mitochondrial function, decreased oxidative damage, and increased metabolic rate

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12 December 2020

Detection of MELAS A3243G point mutation in muscle, blood and hair follicles

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12 December 2020

The mitochondrial tRNA(Leu(UUR)) mutation in mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS): genetic, biochemical, and morphological correlations in skeletal muscle

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12 December 2020

Specific correlation between the wobble modification deficiency in mutant tRNAs and the clinical features of a human mitochondrial disease

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12 December 2020

Acute pancreatitis in an infant with lactic acidosis and a mutation at nucleotide 3243 in the mitochondrial DNA tRNALeu(UUR) gene

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12 December 2020

Effect of phenytoin and corticosteroids on seizures and lipid peroxidation in experimental posttraumatic epilepsy

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12 December 2020

Cytochrome aa3 depletion is the cause of the deficient mitochondrial respiration induced by chronic valproate administration

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12 December 2020

Effects of idebenone and related compounds on respiratory activities of brain mitochondria, and on lipid peroxidation of their membranes

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12 December 2020

Safety of high-dose nicotinamide: a review

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12 December 2020

Successful treatment with succinate in a patient with MELAS.

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12 December 2020

Atypical clinical presentations associated with the MELAS mutation at position 3243 of human mitochondrial DNA

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12 December 2020

Melas: an original case and clinical criteria for diagnosis

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12 December 2020

Corticosteroid treatment of mitochondrial encephalomyopathies

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12 December 2020

A practical approach to the diagnosis and management of MELAS: case report and review

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12 December 2020

A subtype of diabetes mellitus associated with a mutation of mitochondrial DNA.

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12 December 2020

Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS): a correlative study of the clinical features and mitochondrial DNA mutation

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12 December 2020

Mitochondrial encephalomyopathy with corticosteroid dependence

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12 December 2020

CPEO and carnitine deficiency overlapping in MELAS syndrome.

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12 December 2020

Mitochondrial DNA deletion in a patient with mitochondrial myopathy, lactic acidosis, and stroke-like episodes (MELAS) and Fanconi's syndrome.

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12 December 2020

Nutritional cofactor treatment in mitochondrial disorders

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12 December 2020

Dichloroacetate treatment in Leigh syndrome caused by mitochondrial DNA mutation

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12 December 2020

Codon-specific translational defect caused by a wobble modification deficiency in mutant tRNA from a human mitochondrial disease

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12 December 2020

Plasma concentrations and pharmacokinetics of idebenone and its metabolites following single and repeated doses in young patients with mitochondrial encephalomyopathy

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12 December 2020

Assessment of mitochondrial function and control in normal and diseased states

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12 December 2020

The pharmacology of nitric oxide in the peripheral nervous system of blood vessels

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12 December 2020

MELAS exhibits dominant negative effects on mitochondrial RNA processing

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12 December 2020

Dichloroacetate causes toxic neuropathy in MELAS: a randomized, controlled clinical trial

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12 December 2020

Chronic toxicity of dichloroacetate: possible relation to thiamine deficiency in rats

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12 December 2020

In vitro synthesis and posttranslational uptake of cytochrome c into isolated mitochondria: role of a specific addressing signal in the apocytochrome

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12 December 2020

Valproic acid and the liver.

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12 December 2020

10.2165/00023210-200620060-00002

Identifiers

DOI

1 reference

PubMed ID

16734497

Dimensions Publication ID

14 August 2017

0 references

1 reference

PubMed ID

16734497