(Q35135711)

6 August 2017

title

Mice lacking TR4 nuclear receptor develop mitochondrial myopathy with deficiency in complex I. (English)

1 reference

6 August 2017

main subject

mitochondrial myopathy

1 reference

7

Michael P Massett

1 reference

6 August 2017

Su Liu

1

1 reference

6 August 2017

Yi-Fen Lee

2

1 reference

6 August 2017

Samuel Chou

3

1 reference

6 August 2017

Hideo Uno

4

1 reference

6 August 2017

Gonghui Li

5

1 reference

6 August 2017

Paul Brookes

6

1 reference

6 August 2017

Qiao Wu

8

1 reference

6 August 2017

Lu-Min Chen

9

1 reference

6 August 2017

Chawnshang Chang

10

1 reference

6 August 2017

publication date

26 May 2011

1 reference

6 August 2017

published in

Molecular Endocrinology

1 reference

6 August 2017

volume

25

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6 August 2017

issue

8

1 reference

6 August 2017

page(s)

1301-1310

1 reference

Metformin inhibits nuclear receptor TR4-mediated hepatic stearoyl-CoA desaturase 1 gene expression with altered insulin sensitivity.

6 August 2017

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28 July 2018

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28 July 2018

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The TR2 and TR4 orphan nuclear receptors repress Gata1 transcription

28 July 2018

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28 July 2018

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Human CIA30 is involved in the early assembly of mitochondrial complex I and mutations in its gene cause disease

28 July 2018

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Caveolin-1(-/-)- and caveolin-2(-/-)-deficient mice both display numerous skeletal muscle abnormalities, with tubular aggregate formation

28 July 2018

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28 July 2018

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28 July 2018

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A molecular chaperone for mitochondrial complex I assembly is mutated in a progressive encephalopathy

28 July 2018

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Limited exercise capacity in heterozygous manganese superoxide dismutase gene-knockout mice: roles of superoxide anion and nitric oxide

28 July 2018

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Deficits in motor coordination with aberrant cerebellar development in mice lacking testicular orphan nuclear receptor 4.

28 July 2018

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AIF deficiency compromises oxidative phosphorylation

28 July 2018

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28 July 2018

Mitochondrial disorders

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Characterization of Ighmbp2 in motor neurons and implications for the pathomechanism in a mouse model of human spinal muscular atrophy with respiratory distress type 1 (SMARD1)

28 July 2018

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28 July 2018

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28 July 2018

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Large-scale deletion and point mutations of the nuclear NDUFV1 and NDUFS1 genes in mitochondrial complex I deficiency

28 July 2018

1 reference

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28 July 2018

1 reference

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Convergence of two repressors through heterodimer formation of androgen receptor and testicular orphan receptor-4: a unique signaling pathway in the steroid receptor superfamily

28 July 2018

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A calcium signaling defect in the pathogenesis of a mitochondrial DNA inherited oxidative phosphorylation deficiency

28 July 2018

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Nuclear DNA origin of mitochondrial complex I deficiency in fatal infantile lactic acidosis evidenced by transnuclear complementation of cultured fibroblasts

28 July 2018

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28 July 2018

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28 July 2018

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The neurogenic weakness, ataxia and retinitis pigmentosa (NARP) syndrome mtDNA mutation (T8993G) triggers muscle ATPase deficiency and hypocitrullinaemia

28 July 2018

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Induction of the intronic enhancer of the human ciliary neurotrophic factor receptor (CNTFRalpha) gene by the TR4 orphan receptor. A member of steroid receptor superfamily

28 July 2018

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28 July 2018

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Problems with the biochemical diagnosis in mitochondrial (encephalo-)myopathies

28 July 2018

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Deficiency of the reduced nicotinamide adenine dinucleotide dehydrogenase component of complex I of mitochondrial electron transport. Fatal infantile lactic acidosis and hypermetabolism with skeletal-cardiac myopathy and encephalopathy

28 July 2018

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Chapter 9 Reliable assay for measuring complex I activity in human blood lymphocytes and skin fibroblasts.

28 July 2018

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The production of reactive oxygen species by complex I.

27 September 2018

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Assembly of mitochondrial complex I and defects in disease

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3146253

Superoxide production is inversely related to complex I activity in inherited complex I deficiency.

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3146253

Dysfunctions of cellular oxidative metabolism in patients with mutations in the NDUFS1 and NDUFS4 genes of complex I

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3146253

Secondary metabolic effects in complex I deficiency

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3146253

Induction of apolipoprotein E expression by TR4 orphan nuclear receptor via 5' proximal promoter region.

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3146253

Growth retardation and abnormal maternal behavior in mice lacking testicular orphan nuclear receptor 4.

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3146253

Muscle as a putative producer of acid alpha-glucosidase for glycogenosis type II gene therapy

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3146253

CIA30 complex I assembly factor: a candidate for human complex I deficiency?

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3146253

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

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3146253

Molecular genetic aspects of human mitochondrial disorders

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3146253

Ragged red or ragged blue fibers

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3146253

Mitochondrial Membrane Potential and ATP Production in Primary Disorders of ATP Synthase

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3146253

Tubular aggregates in paralysis periodica paramyotonica with T704M mutation of SCN4A

16 November 2018

1 reference

12 December 2020

Muscle weakness in a mouse model of nemaline myopathy can be reversed with exercise and reveals a novel myofiber repair mechanism

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Strain-dependent differences in responses to exercise training in inbred and hybrid mice

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Loss of TR4 Orphan Nuclear Receptor Reduces Phosphoenolpyruvate Carboxykinase Mediated Gluconeogenesis

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Myxovirus-Like Structures in a Case of Human Chronic Polymyositis

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Spectrophotometric Assay for Complex I of the Respiratory Chain in Tissue Samples and Cultured Fibroblasts

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Identifiers

DOI

10.1210/ME.2010-0455

1 reference

6 August 2017

1 reference

6 August 2017

1 reference