(Q36936875)

17 August 2017

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title

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

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17 August 2017

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Morath MA

1

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17 August 2017

Okun JG

2

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17 August 2017

Müller IB

3

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17 August 2017

Sauer SW

4

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17 August 2017

Hörster F

5

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17 August 2017

Hoffmann GF

6

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17 August 2017

Kölker S

7

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17 August 2017

publication date

12 September 2007

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Journal of Inherited Metabolic Disease

17 August 2017

published in

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17 August 2017

volume

31

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17 August 2017

issue

1

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17 August 2017

page(s)

35-43

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

17 August 2017

cites work

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12 December 2020

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

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12 December 2020

Glutathione deficiency in patients with mitochondrial disease: implications for pathogenesis and treatment

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12 December 2020

Evidence of ROS generation by mitochondria in cells with impaired electron transport chain and mitochondrial DNA damage.

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12 December 2020

Propionic acidaemia: clinical, biochemical and therapeutic aspects. Experience in 30 patients

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12 December 2020

Hyperkalemia after acute metabolic decompensation in two children with vitamin B12-unresponsive methylmalonic acidemia and normal renal function

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12 December 2020

Approaches to the treatment of mitochondrial diseases

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12 December 2020

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

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12 December 2020

Type 4 renal tubular acidosis (subtype 2) in a patient with methylmalonic acidaemia

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12 December 2020

Cardiomyopathy in propionic acidaemia

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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.

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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

Deficiency of the ADP-forming succinyl-CoA synthase activity is associated with encephalomyopathy and mitochondrial DNA depletion

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12 December 2020

Neurological outcome of methylmalonic acidaemia

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Current role of liver transplantation for methylmalonic acidemia: a review of the literature

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12 December 2020

Effect of 2-Methylcitrate on Citrate Metabolism: Implications for the Management of Patients with Propionic acidemia and Methylmalonic aciduria

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12 December 2020

Mitochondrial encephalomyopathy with elevated methylmalonic acid is caused by SUCLA2 mutations

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12 December 2020

Pyruvate dehydrogenase, substrate specificity and product inhibition.

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12 December 2020

SUCLA2 mutations are associated with mild methylmalonic aciduria, Leigh-like encephalomyopathy, dystonia and deafness

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12 December 2020

Liver transplantation is not curative for methylmalonic acidopathy caused by methylmalonyl-CoA mutase deficiency

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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

Elevation of intrarenal adenosine by maleic acid decreases GFR and renin release

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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

Detection of maleate-induced Fanconi syndrome by decreasing accumulation of 125I-3-iodo-alpha-methyl-L-tyrosine in the proximal tubule segment-1 region of renal cortex in mice: a trial of separate evaluation of reabsorption

1 reference

12 December 2020

Substrate specificity of the human renal sodium dicarboxylate cotransporter, hNaDC-3, under voltage-clamp conditions

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Microperfusion study of proximal tubule bicarbonate transport in maleic acid-induced renal tubular acidosis

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12 December 2020

Compartmentation of glutathione: implications for the study of toxicity and disease.

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12 December 2020

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

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12 December 2020

Secondary mitochondrial dysfunction in propionic aciduria: a pathogenic role for endogenous mitochondrial toxins.

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12 December 2020

Metabolic stroke in methylmalonic acidemia five years after liver transplantation

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12 December 2020

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

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12 December 2020

Renal tubular dysfunction in methylmalonic acidaemia

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12 December 2020

On the mechanism of action of the antifungal agent propionate.

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12 December 2020

Structure, function, and genomic organization of human Na(+)-dependent high-affinity dicarboxylate transporter

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12 December 2020

Studies of propionate toxicity in Salmonella enterica identify 2-methylcitrate as a potent inhibitor of cell growth

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12 December 2020

Functional and molecular identification of sodium-coupled dicarboxylate transporters in rat primary cultured cerebrocortical astrocytes and neurons

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12 December 2020

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

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12 December 2020

The specific inhibition of the pyruvate dehydrogenase complex from pig kidney by propionyl-CoA and isovaleryl-Co-A

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12 December 2020

Methylmalonic acid--an endogenous toxin?

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12 December 2020

Brain-to-blood transporters for endogenous substrates and xenobiotics at the blood-brain barrier: an overview of biology and methodology

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12 December 2020

Methylmalonate toxicity in primary neuronal cultures

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12 December 2020

Evidence for mitochondrial uptake of glutathione by dicarboxylate and 2-oxoglutarate carriers

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12 December 2020

Molecular properties of sodium/dicarboxylate cotransporters

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Transport of N-acetylaspartate by the Na(+)-dependent high-affinity dicarboxylate transporter NaDC3 and its relevance to the expression of the transporter in the brain

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12 December 2020

Inhibition by propionyl-coenzyme A of N-acetylglutamate synthetase in rat liver mitochondria. A possible explanation for hyperammonemia in propionic and methylmalonic acidemia

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12 December 2020

Transport of organic anions across the basolateral membrane of proximal tubule cells

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12 December 2020

'Classical' organic acidurias, propionic aciduria, methylmalonic aciduria and isovaleric aciduria: long-term outcome and effects of expanded newborn screening using tandem mass spectrometry.

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12 December 2020

Experimental Fanconi syndrome. I. Effect of maleic acid on renal cortical Na-K-ATPase activity and ATP levels

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12 December 2020

Chronic renal failure in methylmalonic acidaemia

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12 December 2020

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

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12 December 2020

The mitochondrial genome in human adaptive radiation and disease: on the road to therapeutics and performance enhancement

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12 December 2020

Liver transplantation for inborn errors of metabolism

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12 December 2020

Lactate dehydrogenase activity is inhibited by methylmalonate in vitro

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12 December 2020

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

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12 December 2020

Pancreatitis in patients with organic acidemias

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12 December 2020

Reversible end-stage renal disease in an adolescent patient with methylmalonic aciduria

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12 December 2020

Lysine intake and neurotoxicity in glutaric aciduria type I: towards a rationale for therapy?

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12 December 2020

Free radicals and antioxidants in normal physiological functions and human disease

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12 December 2020

10.1007/S10545-007-0571-5

Identifiers

DOI

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17 August 2017

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