Metabolic interventions against complex I deficiency in MELAS syndrome (Q73729974)

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6 November 2019

title

Metabolic interventions against complex I deficiency in MELAS syndrome (English)

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6 November 2019

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K Majamaa

1

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6 November 2019

H Rusanen

series ordinal

2

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6 November 2019

A Remes

series ordinal

3

1 reference

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6 November 2019

I E Hassinen

series ordinal

4

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6 November 2019

publication date

1 September 1997

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Molecular and Cellular Biochemistry

6 November 2019

published in

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6 November 2019

volume

174

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6 November 2019

issue

1-2

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6 November 2019

page(s)

291-296

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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.

6 November 2019

cites work

1 reference

https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

Deletion of mitochondrial DNA in a case of early-onset diabetes mellitus, optic atrophy and deafness (DIDMOAD, Wolfram syndrome)

1 reference

https://pubmed.ncbi.nlm.nih.gov/9309702

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

Therapeutic effect of sodium dichloroacetate on visual and auditory hallucinations in a patient with MELAS.

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https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

Relationships between energy level and insulin secretion in isolated rat islets of Langerhans. A study at various pH values

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https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

Diabetes mellitus carrying a mutation in the mitochondrial tRNA(Leu(UUR)) gene

1 reference

https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

A simple method for calculating Km and V from a single enzyme reaction progress curve.

1 reference

https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

State of oxidation-reduction and state of binding in the cytosolic NADH-system as disclosed by equilibration with extracellular lactate-pyruvate in hemoglobin-free perfused rat liver

1 reference

https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

Increase in hepatic NAD level--its effect on the redox state and on ethanol and acetaldehyde metabolism

1 reference

https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

Adult-onset diabetes mellitus and neurosensory hearing loss in maternal relatives of MELAS patients in a family with the tRNA(Leu(UUR)) mutation

1 reference

https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

Mitochondrial membrane potential, transmembrane difference in the NAD+ redox potential and the equilibrium of the glutamate-aspartate translocase in the isolated perfused rat heart

1 reference

https://pubmed.ncbi.nlm.nih.gov/9309702

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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https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

Deficiency of subunits of Complex I and mitochondrial encephalomyopathy

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12 December 2020

Pyridine nucleotide synthesis in the mouse

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https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

Subcellular origin of the surface fluorescence of reduced nicotinamide nucleotides in the isolated perfused rat heart

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https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

Treatment of lactic acidosis with dichloroacetate

1 reference

https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

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

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https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

Treatment of complex I deficiency with riboflavin

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https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

Protection by ascorbic acid against oxidative injury of isolated hepatocytes

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https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

NADH-CoQ reductase deficient myopathy: successful treatment with riboflavin

1 reference

https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

Exogenous coenzyme Q (coq) fails to increase coq in skeletal muscle of two patients with mitochondrial myopathies

1 reference

https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

Evaluation of procedures for assaying oxidative phosphorylation enzyme activities in mitochondrial myopathy muscle biopsies

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https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

Mitochondrial respiratory control in the myocardium

1 reference

https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

Biochemical studies of skeletal muscle mitochondria. I. Microanalysis of cytochrome content, oxidative and phosphorylative activities of mammalian skeletal muscle mitochondria

1 reference

https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

Photolabelling of a mitochondrially encoded subunit of NADH dehydrogenase with [3H]dihydrorotenone.

1 reference

https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

The metabolism of menadione (2-methyl-1,4-naphthoquinone) by isolated hepatocytes. A study of the implications of oxidative stress in intact cells

1 reference

https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

Mutation in mitochondrial tRNALeu(UUR) gene in a large pedigree with maternally transmitted type II diabetes mellitus and deafness

1 reference

https://pubmed.ncbi.nlm.nih.gov/9309702

12 December 2020

Identifiers

DOI

10.1023/A:1006833200371

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:9309702%20AND%20SRC:MED&resulttype=core&format=json

Dimensions Publication ID

6 November 2019

1020993630

0 references

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