Altered mitochondrial function in fibroblasts containing MELAS or MERRF mitochondrial DNA mutations. (Q42077715)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:8809026%20AND%20SRC:MED&resulttype=core&format=json

19 October 2019

title

Altered mitochondrial function in fibroblasts containing MELAS or MERRF mitochondrial DNA mutations (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:8809026%20AND%20SRC:MED&resulttype=core&format=json

19 October 2019

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1 reference

2

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19 October 2019

author name string

A M James

series ordinal

1

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19 October 2019

C Y Pang

series ordinal

3

1 reference

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19 October 2019

M P Murphy

series ordinal

4

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19 October 2019

language of work or name

English

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publication date

1 September 1996

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19 October 2019

number of pages

7

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inferred from page(s)

published in

Biochemical Journal

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19 October 2019

volume

318 ( Pt 2)

1 reference

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19 October 2019

page(s)

401-407

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Neuropathology of mitochondrial encephalomyopathies due to mitochondrial DNA defects.

19 October 2019

cites work

1 reference

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A microtiter plate assay for cytochrome c oxidase in permeabilized whole cells

30 May 2018

1 reference

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MELAS syndrome with mitochondrial tRNA(Leu(UUR)) gene mutation in a Chinese family

30 May 2018

1 reference

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Mitochondrial DNA sequence variation in human evolution and disease.

30 May 2018

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Mitochondrial calcium transport: physiological and pathological relevance

30 May 2018

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Mitochondrial DNA alterations and genetic diseases: a review

30 May 2018

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Abnormal calcium homeostasis and mitochondrial polarization in a human encephalomyopathy

30 May 2018

1 reference

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1994 William Allan Award Address. Mitochondrial DNA variation in human evolution, degenerative disease, and aging

30 May 2018

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Sequence and organization of the human mitochondrial genome

30 May 2018

1 reference

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O2 solubility in aqueous media determined by a kinetic method.

30 May 2018

1 reference

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Measurement of protein using bicinchoninic acid

30 May 2018

1 reference

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Control of electron flux through the respiratory chain in mitochondria and cells

30 May 2018

1 reference

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Thermodynamic control of electron flux through mitochondrial cytochrome bc1 complex

30 May 2018

1 reference

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Human cells lacking mtDNA: repopulation with exogenous mitochondria by complementation

30 May 2018

1 reference

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Non-ohmic proton conductance of the mitochondrial inner membrane in hepatocytes.

30 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1217636

A point mutation in the mitochondrial tRNA(Leu)(UUR) gene in MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes).

30 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1217636

Myoclonic epilepsy and ragged-red fiber disease (MERRF) is associated with a mitochondrial DNA tRNA(Lys) mutation

30 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1217636

A mutation in the tRNA(Leu)(UUR) gene associated with the MELAS subgroup of mitochondrial encephalomyopathies.

30 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1217636

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

30 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1217636

MELAS mutation in mtDNA binding site for transcription termination factor causes defects in protein synthesis and in respiration but no change in levels of upstream and downstream mature transcripts

30 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1217636

Mitochondrial genetics: a paradigm for aging and degenerative diseases?

30 May 2018

1 reference

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The mitochondrial tRNA(Leu)(UUR)) mutation in MELAS: a model for pathogenesis

30 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1217636

7 Preparation and properties of dihydroubiquinone: Cytochrome c oxidoreductase (complex III)

30 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1217636

Oxidation of ferrocytochrome c by mitochondrial cytochrome c oxidase

30 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1217636

Myoclonic epilepsy with ragged-red fibers (MERRF) syndrome: report of a Chinese family with mitochondrial DNA point mutation in tRNA(Lys) gene.

30 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1217636

Lack of major mitochondrial bioenergetic changes in cultured skin fibroblasts from aged individuals.

17 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1217636

MtDNA mutation in MERRF syndrome causes defective aminoacylation of tRNA(Lys) and premature translation termination

17 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1217636

Mitochondrial DNA mutation in a Chinese family with myoclonic epilepsy and ragged-red fiber disease

17 August 2018

1 reference

12 December 2020

Preparation and properties of succinate: ubiquinone oxidoreductase (complex II)

1 reference

12 December 2020

Monitoring of the mitochondrial and plasma membrane potentials in human fibroblasts by tetraphenylphosphonium ion distribution

1 reference

12 December 2020

Uridine preserves the expression of respiratory enzyme deficiencies in cultured fibroblasts

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12 December 2020

Assay conditions for the mitochondrial NADH:coenzyme Q oxidoreductase

1 reference

12 December 2020

Identifiers

DOI

10.1042/BJ3180401

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:8809026%20AND%20SRC:MED&resulttype=core&format=json

19 October 2019

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:8809026%20AND%20SRC:MED&resulttype=core&format=json

19 October 2019

1 reference