Metabolite profiles reveal energy failure and impaired beta-oxidation in liver of mice with complex III deficiency due to a BCS1L mutation (Q31076216)

14 October 2017

Metabolite profiles reveal energy failure and impaired beta-oxidation in liver of mice with complex III deficiency due to a BCS1L mutation (English)

1 reference

14 October 2017

Heike Kotarsky

1

1 reference

14 October 2017

Matthias Keller

2

1 reference

14 October 2017

Mina Davoudi

3

1 reference

14 October 2017

Per Levéen

4

1 reference

14 October 2017

Riitta Karikoski

5

1 reference

14 October 2017

David P Enot

6

1 reference

14 October 2017

Vineta Fellman

7

1 reference

14 October 2017

language of work or name

English

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publication date

19 July 2012

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14 October 2017

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14 October 2017

7

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14 October 2017

7

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14 October 2017

e41156

1 reference

Creative Commons Attribution 4.0 International

14 October 2017

start time

19 July 2012

1 reference

April 2022 Public Data File from Crossref

copyright status

copyrighted

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cites work

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The GRACILE mutation introduced into Bcs1l causes postnatal complex III deficiency: a viable mouse model for mitochondrial hepatopathy

21 June 2018

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21 June 2018

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21 June 2018

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21 June 2018

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SIRT3 regulates mitochondrial fatty-acid oxidation by reversible enzyme deacetylation

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PGC-1alpha, SIRT1 and AMPK, an energy sensing network that controls energy expenditure

21 June 2018

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Role of phospholipids in respiratory cytochrome bc(1) complex catalysis and supercomplex formation

21 June 2018

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Respiratory-chain diseases related to complex III deficiency

21 June 2018

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Cytochrome c oxidase deficiency in neurons decreases both oxidative stress and amyloid formation in a mouse model of Alzheimer's disease

21 June 2018

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Missense mutations in the BCS1L gene as a cause of the Björnstad syndrome

21 June 2018

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21 June 2018

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21 June 2018

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21 June 2018

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Molecular mediators of hepatic steatosis and liver injury

21 June 2018

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21 June 2018

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21 June 2018

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21 June 2018

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GRACILE syndrome, a lethal metabolic disorder with iron overload, is caused by a point mutation in BCS1L

21 June 2018

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A mutant mitochondrial respiratory chain assembly protein causes complex III deficiency in patients with tubulopathy, encephalopathy and liver failure.

21 June 2018

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Bcs1p, an AAA-family member, is a chaperone for the assembly of the cytochrome bc(1) complex.

21 June 2018

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21 June 2018

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21 June 2018

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Further insights into the assembly of the yeast cytochrome bc1 complex based on analysis of single and double deletion mutants lacking supernumerary subunits and cytochrome b.

21 June 2018

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Cellular pathophysiological consequences of BCS1L mutations in mitochondrial complex III enzyme deficiency.

21 January 2018

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Metabolomics (liver and blood profiling) in a mouse model in response to fasting: a study of hepatic steatosis

21 January 2018

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Oxygen sensing requires mitochondrial ROS but not oxidative phosphorylation

21 January 2018

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Tissue-, substrate-, and site-specific characteristics of mitochondrial reactive oxygen species generation

21 January 2018

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Increased levels of cell-free hemoglobin, oxidation markers, and the antioxidative heme scavenger alpha(1)-microglobulin in preeclampsia

21 January 2018

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Neonatal Fanconi syndrome due to deficiency of complex III of the respiratory chain

21 January 2018

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Protein Carbonyl Measurement by a Sensitive ELISA Method

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Exercise intolerance due to mutations in the cytochrome b gene of mitochondrial DNA

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Metabolomic analysis of sulfur-containing substances and polyamines in regenerating rat liver

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12 December 2020

10.1371/JOURNAL.PONE.0041156

Identifiers

DOI

1 reference

14 October 2017

0 references

1 reference

14 October 2017

PubMed publication ID

22829922

1 reference