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Literature DB >> 4398537

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

Abstract

We report the effects of methylmalonic acid (MMA) on the mitochondrial transport systems for malate, alpha-oxoglutarate, and isocitrate. MMA is shown to be a substrate for all three carrier systems, and an inhibitor of the malate-phosphate exchange carrier. The effects of MMA on the metabolism of malate, oxoglutarate, and isocitrate by rat liver mitochondria are demonstrated to be mediated by the influence of MMA on the transport step. A hypothesis regarding the metabolic impairments responsible for hypoglycemia and ketonemia in methylmalonic aciduria is formulated in relation to these findings.

Entities: Chemical Disease Species

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Year: 1971 PMID： 4398537 PMCID： PMC292169 DOI： 10.1172/JCI106725

Source DB: PubMed Journal: J Clin Invest ISSN： 0021-9738 Impact factor: 14.808

17 in total

1. The inhibition of malate, tricarboxylate and oxoglutarate entry into mitochondria by 2-n-butylmalonate.

Authors: B H Robinson; J B Chappell
Journal: Biochem Biophys Res Commun Date: 1967-07-21 Impact factor: 3.575

2. Systems used for the transport of substrates into mitochondria.

Authors: J B Chappell
Journal: Br Med Bull Date: 1968-05 Impact factor: 4.291

3. Methylmalonic aciduria. An inborn error leading to metabolic acidosis, long-chain ketonuria and intermittent hyperglycinemia.

Authors: L E Rosenberg; A C Lilljeqvist; Y E Hsia
Journal: N Engl J Med Date: 1968-06-13 Impact factor: 91.245

4. Methylmalonic acidemia. A disorder associated with acidosis, hyperglycinemia, and hyperlactatemia.

Authors: B Lindblad; B S Lindblad; P Olin; B Svanberg; R Zetterström
Journal: Acta Paediatr Scand Date: 1968-09

5. Studies in a patient with methylmalonic acidemia.

Authors: G Morrow; L A Barness
Journal: J Pediatr Date: 1969-05 Impact factor: 4.406

6. A derangement in B 12 metabolism leading to homocystinemia, cystathioninemia and methylmalonic aciduria.

Authors: S H Mudd; H L Levy; R H Abeles; J P Jennedy
Journal: Biochem Biophys Res Commun Date: 1969-04-10 Impact factor: 3.575

7. Observations on the coexistence of methylmalonic acidemia and glycinemia.

Authors: G Morrow; L A Barness; V H Auerbach; A M DiGeorge; T Ando; W L Nyhan
Journal: J Pediatr Date: 1969-05 Impact factor: 4.406

8. Paths of carbon in gluconeogenesis and lipogenesis: the role of mitochondria in supplying precursors of phosphoenolpyruvate.

Authors: H A Lardy; V Paetkau; P Walter
Journal: Proc Natl Acad Sci U S A Date: 1965-06 Impact factor: 11.205

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

Authors: V G Oberholzer; B Levin; E A Burgess; W F Young
Journal: Arch Dis Child Date: 1967-10 Impact factor: 3.791

10. Congenital methylmalonic acidemia: enzymatic evidence for two forms of the disease.

Authors: G Morrow; L A Barness; G J Cardinale; R H Abeles; J G Flaks
Journal: Proc Natl Acad Sci U S A Date: 1969-05 Impact factor: 11.205

18 in total

Review 1. Mitochondrial energy metabolism in neurodegeneration associated with methylmalonic acidemia.

Authors: Daniela R Melo; Alicia J Kowaltowski; Moacir Wajner; Roger F Castilho
Journal: J Bioenerg Biomembr Date: 2011-02 Impact factor: 2.945

2. Lactate dehydrogenase activity is inhibited by methylmalonate in vitro.

Authors: Laura O Saad; Sandra R Mirandola; Evelise N Maciel; Roger F Castilho
Journal: Neurochem Res Date: 2006-05-09 Impact factor: 3.996

3. Reversible dysregulation of the mitochondrial ATP synthase in skin fibroblasts from children with vitamin B12-dependent methylmalonic acidaemia.

Authors: A M Das
Journal: J Inherit Metab Dis Date: 1997-07 Impact factor: 4.982

Review 4. Metabolic disturbances in diseases with neurological involvement.

Authors: João M N Duarte; Patrícia F Schuck; Gary L Wenk; Gustavo C Ferreira
Journal: Aging Dis Date: 2013-11-30 Impact factor: 6.745

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

Authors: P Narasimhan; R Sklar; M Murrell; R A Swanson; F R Sharp
Journal: J Neurosci Date: 1996-11-15 Impact factor: 6.167

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

Authors: J C Dutra; C S Dutra-Filho; S E Cardozo; C M Wannmacher; J J Sarkis; M Wajner
Journal: J Inherit Metab Dis Date: 1993 Impact factor: 4.982

10. Failure of the normal ureagenic response to amino acids in organic acid-loaded rats. Proposed mechanism for the hyperammonemia of propionic and methylmalonic acidemia.

Authors: P M Stewart; M Walser
Journal: J Clin Invest Date: 1980-09 Impact factor: 14.808