Methylmalonic Acid, a Biochemical Hallmark of Methylmalonic Acidurias but No Inhibitor of Mitochondrial Respiratory Chain
Open Access
- 1 November 2003
- journal article
- Published by Elsevier in Journal of Biological Chemistry
- Vol. 278 (48) , 47388-47393
- https://doi.org/10.1074/jbc.m308861200
Abstract
No abstract availableKeywords
This publication has 30 references indexed in Scilit:
- Inhibition of the mitochondrial respiratory chain complex activities in rat cerebral cortex by methylmalonic acidNeurochemistry International, 2002
- Ascorbic acid and α-tocopherol attenuate methylmalonic acid-induced convulsionsNeuroReport, 1999
- Role of Deprotonation Events in Ubihydroquinone:Cytochrome c Oxidoreductase from Bovine Heart and Yeast MitochondriaBiochemistry, 1997
- Intrastriatal methylmalonic acid administration induces rotational behavior and convulsions through glutamatergic mechanismsBrain Research, 1996
- Clinical outcome of long-term management of patients with vitamin B12-unresponsive methylmalonic acidemiaThe Journal of Pediatrics, 1994
- Inhibition of Succinate Dehydrogenase by Malonic Acid Produces an “Excitotoxic” Lesion in Rat StriatumJournal of Neurochemistry, 1993
- Acute extrapyramidal syndrome in methylmalonic acidemia: “Metabolic stroke” involving the globus pallidusThe Journal of Pediatrics, 1988
- Investigation of mitochondrial metabolism in small human skeletal muscle biopsy specimens. Improvement of preparation procedureClinica Chimica Acta; International Journal of Clinical Chemistry, 1985
- The inhibition by methylmalonic acid of malate transport by the dicarboxylate carrier in rat liver mitochondriaJournal of Clinical Investigation, 1971
- Methylmalonic aciduria. An inborn error of metabolism leading to chronic metabolic acidosis.Archives of Disease in Childhood, 1967