(Q42447507)

31 October 2017

Methylmalonic acid, a biochemical hallmark of methylmalonic acidurias but no inhibitor of mitochondrial respiratory chain (English)

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31 October 2017

6

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31 October 2017

Stefan Kölker

1

1 reference

31 October 2017

Marina Schwab

2

1 reference

31 October 2017

Friederike Hörster

3

1 reference

31 October 2017

Sven Sauer

4

1 reference

31 October 2017

Angela Hinz

5

1 reference

31 October 2017

Ertan Mayatepek

7

1 reference

31 October 2017

Georg F Hoffmann

8

1 reference

31 October 2017

Jan A M Smeitink

9

1 reference

31 October 2017

Jürgen G Okun

10

1 reference

31 October 2017

language of work or name

English

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publication date

12 September 2003

1 reference

Journal of Biological Chemistry

31 October 2017

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31 October 2017

278

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31 October 2017

47388-47393

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31 October 2017

48

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Methylmalonicacidemia: biochemical heterogeneity in defects of 5'-deoxyadenosylcobalamin synthesis

31 October 2017

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M308861200

Clinical outcome of long-term management of patients with vitamin B12-unresponsive methylmalonic acidemia

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M308861200

Methylmalonic acidaemia with bilateral globus pallidus involvement: a neuropathological study.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M308861200

Defect in succinate oxidation by isolated muscle mitochondria in a patient with symmetrical lesions in the basal ganglia

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M308861200

Inhibition of succinate dehydrogenase and beta-hydroxybutyrate dehydrogenase activities by methylmalonate in brain and liver of developing rats.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M308861200

Inhibition of the mitochondrial respiratory chain complex activities in rat cerebral cortex by methylmalonic acid.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M308861200

Neurological dysfunction in methylmalonic acidaemia is probably related to the inhibitory effect of methylmalonate on brain energy production

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M308861200

Alternative excitotoxic hypotheses

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M308861200

Three classes of inhibitors share a common binding domain in mitochondrial complex I (NADH:ubiquinone oxidoreductase).

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M308861200

3-Ureidopropionate contributes to the neuropathology of 3-ureidopropionase deficiency and severe propionic aciduria: a hypothesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M308861200

Investigation of mitochondrial metabolism in small human skeletal muscle biopsy specimens. Improvement of preparation procedure.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M308861200

Disorders of the mitochondrial respiratory chain: clinical manifestations and diagnostic approach

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M308861200

The inhibition by methylmalonic acid of malate transport by the dicarboxylate carrier in rat liver mitochondria. A possible explantation for hypoglycemia in methylmalonic aciduria

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M308861200

Methylmalonic aciduria. An inborn error of metabolism leading to chronic metabolic acidosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M308861200

Neurodegeneration in methylmalonic aciduria involves inhibition of complex II and the tricarboxylic acid cycle, and synergistically acting excitotoxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M308861200

Role of Deprotonation Events in Ubihydroquinone:CytochromecOxidoreductase from Bovine Heart and Yeast Mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M308861200

Acute extrapyramidal syndrome in methylmalonic acidemia: “Metabolic stroke” involving the globus pallidus

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M308861200

Methylmalonic acid induces excitotoxic neuronal damage in vitro

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M308861200

Intrastriatal methylmalonic acid administration induces rotational behavior and convulsions through glutamatergic mechanisms

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M308861200

Ascorbic acid and alpha-tocopherol attenuate methylmalonic acid-induced convulsions

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M308861200

Estimation of energy metabolism in human skeletal muscle homogenate as a diagnostic aid

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M308861200

Inhibition of succinate dehydrogenase by malonic acid produces an "excitotoxic" lesion in rat striatum

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M308861200

Methylmalonate administration decreases Na+,K+-ATPase activity in cerebral cortex of rats

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M308861200

21 January 2018

Identifiers

DOI

10.1074/JBC.M308861200

1 reference

31 October 2017

1 reference