(Q53675259)

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Mitochondrial network complexity and pathological decrease in complex I activity are tightly correlated in isolated human complex I deficiency.

scientific article published on 18 May 2005

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scholarly article

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Europe PubMed Central

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15901599%20AND%20SRC:MED&resulttype=core&format=json

11 January 2020

Mitochondrial network complexity and pathological decrease in complex I activity are tightly correlated in isolated human complex I deficiency (English)

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Europe PubMed Central

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15901599%20AND%20SRC:MED&resulttype=core&format=json

11 January 2020

Peter H Willems

6

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11 January 2020

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Werner J H Koopman

1

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11 January 2020

Henk-Jan Visch

2

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11 January 2020

Sjoerd Verkaart

3

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11 January 2020

Lambertus W P J van den Heuvel

4

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11 January 2020

Jan A M Smeitink

5

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11 January 2020

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18 May 2005

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11 January 2020

American Journal of Physiology - Cell Physiology

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11 January 2020

289

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11 January 2020

4

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11 January 2020

C881-90

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11 January 2020

OPA1, encoding a dynamin-related GTPase, is mutated in autosomal dominant optic atrophy linked to chromosome 3q28

1 reference

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Percolation and criticality in a mitochondrial network.

1 reference

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Clinical heterogeneity in patients with mutations in the NDUFS4 gene of mitochondrial complex I

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Status of mitochondria in living human fibroblasts during growth and senescence in vitro: use of the laser dye rhodamine 123

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Cytochrome C-mediated apoptosis

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Regulation of mitochondrial ATP synthesis by calcium: evidence for a long-term metabolic priming

1 reference

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NDUFS6 mutations are a novel cause of lethal neonatal mitochondrial complex I deficiency

1 reference

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21 January 2018

Membrane-initiated Ca(2+) signals are reshaped during propagation to subcellular regions

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21 January 2018

Inhibition of complex I of the electron transport chain causes O2-. -mediated mitochondrial outgrowth.

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21 January 2018

Recombinant expression of the Ca(2+)-sensitive aspartate/glutamate carrier increases mitochondrial ATP production in agonist-stimulated Chinese hamster ovary cells

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21 January 2018

The first nuclear-encoded complex I mutation in a patient with Leigh syndrome

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21 January 2018

Isolated complex I deficiency in children: clinical, biochemical and genetic aspects

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21 January 2018

Mutations in the complex I NDUFS2 gene of patients with cardiomyopathy and encephalomyopathy

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21 January 2018

Excessive formation of hydroxyl radicals and aldehydic lipid peroxidation products in cultured skin fibroblasts from patients with complex I deficiency

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21 January 2018

Disruption of mitochondrial networks by the human cytomegalovirus UL37 gene product viral mitochondrion-localized inhibitor of apoptosis

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21 January 2018

Superoxide activates uncoupling proteins by generating carbon-centered radicals and initiating lipid peroxidation: studies using a mitochondria-targeted spin trap derived from alpha-phenyl-N-tert-butylnitrone.

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21 January 2018

Altered mitochondrial structure and motion dynamics in living cells with energy metabolism defects revealed by real time microscope imaging

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Energy substrate modulates mitochondrial structure and oxidative capacity in cancer cells

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21 January 2018

Mutant NDUFV1 subunit of mitochondrial complex I causes leukodystrophy and myoclonic epilepsy

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Mitochondrial filaments and clusters as intracellular power-transmitting cables

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Human mitochondrial complex I in health and disease

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Human NADH:ubiquinone oxidoreductase

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The genetics and pathology of oxidative phosphorylation

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Active mitochondria surrounding the pancreatic acinar granule region prevent spreading of inositol trisphosphate-evoked local cytosolic Ca(2+) signals.

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Leigh syndrome associated with a mutation in the NDUFS7 (PSST) nuclear encoded subunit of complex I.

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Human complex I defects can be resolved by monoclonal antibody analysis into distinct subunit assembly patterns

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Human mitochondrial complex I assembles through the combination of evolutionary conserved modules: a framework to interpret complex I deficiencies.

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Demonstration of a new pathogenic mutation in human complex I deficiency: a 5-bp duplication in the nuclear gene encoding the 18-kD (AQDQ) subunit

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Functional and morphological abnormalities of mitochondria harbouring the tRNA(Leu)(UUR) mutation in mitochondrial DNA derived from patients with maternally inherited diabetes and deafness (MIDD) and progressive kidney disease.

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Human mitochondrial complex I deficiency: investigating transcriptional responses by microarray.

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Inhibition of mitochondrial Na+-Ca2+ exchange restores agonist-induced ATP production and Ca2+ handling in human complex I deficiency.

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