Mitochondrial energy metabolism in neurodegeneration associated with methylmalonic acidemia (Q37832681)

24 August 2017

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title

Mitochondrial energy metabolism in neurodegeneration associated with methylmalonic acidemia (English)

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24 August 2017

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2

Alicia J Kowaltowski

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24 August 2017

3

Moacir Wajner

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24 August 2017

Daniela R Melo

1

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PubMed ID

21271280

24 August 2017

Roger F Castilho

series ordinal

4

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24 August 2017

1 February 2011

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Journal of Bioenergetics and Biomembranes

24 August 2017

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24 August 2017

43

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24 August 2017

39-46

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24 August 2017

1

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https://scigraph.springernature.com/pub.10.1007/s10863-011-9330-2

24 August 2017

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cites work

Basal ganglia neurotoxins

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Alternative excitotoxic hypotheses

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Opening of mitochondrial K+ channels increases ischemic ATP levels by preventing hydrolysis

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

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/10.1007%2FS10863-011-9330-2

Genetic and genomic systems to study methylmalonic acidemia.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Metabolic phenotype of methylmalonic acidemia in mice and humans: the role of skeletal muscle

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Mitochondrial dysfunction in mut methylmalonic acidemia

21 January 2018

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https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Effects of acute and chronic administration of methylmalonic and propionic acids on the in vitro incorporation of 32P into cytoskeletal proteins from cerebral cortex of young rats.

21 January 2018

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https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Methylmalonic and propionic aciduria

21 January 2018

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https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Effects of methylmalonate and propionate on uptake of glucose and ketone bodies in vitro by brain of developing rats.

21 January 2018

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https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

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/10.1007%2FS10863-011-9330-2

Mitochondrial ATP-sensitive K+ channels prevent oxidative stress, permeability transition and cell death.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Purification and properties of methylmalonyl coenzyme A mutase from human liver

21 January 2018

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https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

GM1 ganglioside attenuates convulsions and thiobarbituric acid reactive substances production induced by the intrastriatal injection of methylmalonic acid

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Propionic and L-methylmalonic acids induce oxidative stress in brain of young rats.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Acute basal ganglia infarction in propionic acidemia

21 January 2018

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https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

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/10.1007%2FS10863-011-9330-2

Looking beyond the basal ganglia: the spectrum of MRI changes in methylmalonic acidaemia

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Cerebral dicarboxylate transport and metabolism studied with isotopically labelled fumarate, malate and malonate

21 January 2018

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https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Intercellular metabolic compartmentation in the brain: past, present and future

21 January 2018

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https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Physiology and pathophysiology of organic acids in cerebrospinal fluid

21 January 2018

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https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Long-term outcome in methylmalonic acidurias is influenced by the underlying defect (mut0, mut-, cblA, cblB).

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Methylmalonyl-CoA mutase (EC 5.4.99.2) and methionine synthetase (EC 2.1.1.13) in the tissues of cobalt-vitamin B12 deficient sheep

21 January 2018

2 references

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

21 January 2018

12 December 2020

Methylmalonic acid--an endogenous toxin?

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

The aetiology of neurological complications of organic acidaemias--a role for the blood-brain barrier.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Diazoxide protects against methylmalonate-induced neuronal toxicity.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Mitochondria and reactive oxygen species

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Mitochondrial glutathione transport: physiological, pathological and toxicological implications.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

The management and outcome of propionic and methylmalonic acidaemia

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Mitochondrial permeability transition in neuronal damage promoted by Ca2+ and respiratory chain complex II inhibition

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Intrastriatal methylmalonic acid administration induces convulsions and TBARS production, and alters Na+,K+-ATPase activity in the rat striatum and cerebral cortex.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Methylmalonate toxicity in primary neuronal cultures

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Methylmalonate inhibits succinate-supported oxygen consumption by interfering with mitochondrial succinate uptake

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Neurodegeneration and chronic renal failure in methylmalonic aciduria--a pathophysiological approach

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Ion-abrasion scanning electron microscopy reveals distorted liver mitochondrial morphology in murine methylmalonic acidemia

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Impaired energy metabolism and abnormal muscle histology in mut- methylmalonic aciduria

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Effect of methylmalonate on ketone body metabolism in developing rat brain.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

A knock-out mouse model for methylmalonic aciduria resulting in neonatal lethality

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Ascorbic acid prevents water maze behavioral deficits caused by early postnatal methylmalonic acid administration in the rat.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Differential inhibitory effects of methylmalonic acid on respiratory chain complex activities in rat tissues

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Assessing the reversibility of the anaplerotic reactions of the propionyl-CoA pathway in heart and liver

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Methylmalonic acidaemia leads to increased production of reactive oxygen species and induction of apoptosis through the mitochondrial/caspase pathway

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Creatine protects against the convulsive behavior and lactate production elicited by the intrastriatal injection of methylmalonate.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Lactate dehydrogenase activity is inhibited by methylmalonate in vitro.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Intracerebral accumulation of glutaric and 3-hydroxyglutaric acids secondary to limited flux across the blood-brain barrier constitute a biochemical risk factor for neurodegeneration in glutaryl-CoA dehydrogenase deficiency.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Impact of short- and medium-chain organic acids, acylcarnitines, and acyl-CoAs on mitochondrial energy metabolism

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Long-term exposure of human proximal tubule cells to hydroxycobalamin[c-lactam] as a possible model to study renal disease in methylmalonic acidurias

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Glutaric aciduria type I and methylmalonic aciduria: simulation of cerebral import and export of accumulating neurotoxic dicarboxylic acids in in vitro models of the blood-brain barrier and the choroid plexus

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Mitochondrial creatine kinase in human health and disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Inhibition of mitochondrial creatine kinase activity from rat cerebral cortex by methylmalonic acid.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Transporter-mediated permeation of drugs across the blood-brain barrier

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Methylmalonic acidemia (MMA).

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Multiple OXPHOS deficiency in the liver of a patient with CblA methylmalonic aciduria sensitive to vitamin B(12).

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

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/10.1007%2FS10863-011-9330-2

Diminished concentrations of ganglioside N-acetylneuraminic acid (G-NeuAc) in cerebellum of young rats receiving chronic administration of methylmalonic acid.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Effect of methylmalonate on in vitro lactate release and carbon dioxide production by brain of suckling rats

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

The role of oxidative damage in the neuropathology of organic acidurias: insights from animal studies.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

Pyruvate carboxylase activity in primary cultures of astrocytes and neurons.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10863-011-9330-2

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

21 January 2018

1 reference

12 December 2020

Long-term follow-up of 77 patients with isolated methylmalonic acidaemia

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12 December 2020

Recognition, isolation, and characterization of rat liver D-methylmalonyl coenzyme A hydrolase

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12 December 2020

Deficiency in complex II of the respiratory chain, presenting as a leukodystrophy in two sisters with Leigh syndrome.

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12 December 2020

Methylmalonic acid inhibits respiration in rat liver mitochondria

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12 December 2020

Multi-slice proton MR spectroscopy and diffusion-weighted imaging in methylmalonic acidemia: report of two cases and review of the literature.

1 reference

12 December 2020

Ionic dependence of glutamate neurotoxicity

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12 December 2020

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

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12 December 2020

Methylmalonyl-CoA mutase induction by cerebral ischemia and neurotoxicity of the mitochondrial toxin methylmalonic acid.

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12 December 2020

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

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12 December 2020

Bioenergetic consequences of opening the ATP-sensitive K(+) channel of heart mitochondria

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12 December 2020

Multiple OXPHOS deficiency in the liver, kidney, heart, and skeletal muscle of patients with methylmalonic aciduria and propionic aciduria

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12 December 2020

A possible role for acetyl coa in the control of gluconeogenesis

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12 December 2020

Massachusetts Metabolic Disorders Screening Program. II. Methylmalonic aciduria

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12 December 2020

Mitochondria and neuronal survival

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12 December 2020

Permeabilization of the inner mitochondrial membrane by Ca2+ ions is stimulated by t-butyl hydroperoxide and mediated by reactive oxygen species generated by mitochondria

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12 December 2020

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

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12 December 2020

Disruption of mitochondrial homeostasis in organic acidurias: insights from human and animal studies

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12 December 2020

Glutathione deficiency as a complication of methylmalonic acidemia: Response to high doses of ascorbate

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12 December 2020

Decreased activities of ubiquinol:ferricytochrome c oxidoreductase (complex III) and ferrocytochrome c:oxygen oxidoreductase (complex IV) in liver mitochondria from rats with hydroxycobalamin[c-lactam]-induced methylmalonic aciduria

1 reference

12 December 2020

CT and MR of the brain in disorders of the propionate and methylmalonate metabolism

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12 December 2020

Comparison of cytosolic and mitochondrial enzyme alterations in the livers of propionic or methylmalonic acidemia: a reduction of cytochrome oxidase activity

1 reference

12 December 2020

10.1007/S10863-011-9330-2

Identifiers

DOI

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24 August 2017

1 reference

PubMed ID

21271280