Methylmalonic acid (MMA)
The inhibition by methylmalonic acid of malate transport by the dicarboxylate carrier in rat liver mitochondria: A possible explanation for hypoglycemia in methylmalonic aciduria
... of methylmalonic acid (MMA) on the mitochondrial transport systems for malate, a-oxoglutarate, and ...isocitrate. MMA is shown to be a substrate for all three carrier systems, and an inhibitor ...
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METHYLMALONIC ACID
... METHYLMALONIC ACID: Borden Award Address. http://pediatrics.aappublications.org/content/51/6/1012[r] ...
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Assay of methylmalonic acid in the serum of patients with cobalamin deficiency using capillary gas chromatography mass spectrometry
... of methylmalonic acid while free of hematologic and neurologic ...methylmalonic acid. We conclude that the ability to measure methylmalonic acid in human serum will be useful in ...
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Population prevalence, attributable risk, and attributable risk percentage for high methylmalonic acid concentrations in the post-folic acid fortification period in the US
... serum MMA compared to those in the younger age group ( P < ...serum MMA in older persons is kid- ney ...high MMA in those with serum creatinine ≥ 130 μ mol/L is ≈ 33% (93/279) com- pared to ≈ 7% ...
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Metabolism of methylmalonic acid in rats Is methylmalonyl coenzyme a racemase deficiency symptomatic in man?
... exchanged acid with spontaneous racemization (relative to the 13C-carboxyl group), followed by reacylation, thus exposing the labeled carboxyl to ...free MMA is that racemization of methylmalonyl (MM)-CoA ...
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A Common Polymorphism in HIBCH Influences Methylmalonic Acid Concentrations in Blood Independently of Cobalamin
... reversible MMA shunt to provide R-MMACoA for the MUT reaction through the deacetylation of S-MMACoA and subsequent reacetylation of the free R-MMA, as described by Mont- gomery et ...
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New developments in the treatment of hyperammonemia: emerging use of carglumic acid
... of methylmalonic acid in blood. In addition propionic acid and its metabolites are found in the ...by MMA and hyperammonemia (805 µ ...
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Vitamin B12 and folate levels in healthy Swiss senior citizens: a prospective study evaluating reference intervals and decision limits
... (holoTC), methylmalonic acid (MMA), homocysteine (Hcy), serum folate, red blood cell (RBC) folate were ...0.001), MMA (nmol/L) 207, 221 and 244 (p < 0.001). Hcy and MMA (both p < ...
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Systematic literature review and meta-analysis on the epidemiology of methylmalonic acidemia (MMA) with a focus on MMA caused by methylmalonyl-CoA mutase (mut) deficiency
... of methylmalonic acid. Isolated MMA is primarily caused by the deficiency of methylmalonyl-CoA mutase (MMA mut; EC ...on MMA with a focus on the MMA mut subtype (OMIM ...of ...
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Rapid healing of a patient with dramatic subacute combined degeneration of spinal cord: a case report
... Case presentation: A 57 year-old Caucasian man suffering from dramatic myelosis of the cervical posterior columns. Apart from associated neurological symptoms (tactile hypaesthesia, reduced vibration sensation, loss of ...
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HYPERGLYCINEMIA WITH KETOSIS DUE TO A DEFECT IN ISOLEUCINE METABOLISM: A PRELIMINARY REPORT
... Fibroblast incubation studies demon- strated normal CO2 production from la- belled propionic acid and methylmalonic acid but defective CO2 production from la- belled isoleucine, placing [r] ...
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Prenatal Diagnosis of Methylmalonic Aciduria
... Quantitation of the abnormal methylmalonic acid peak showed the concentration to be 9 .sgs/ml compared with a normal range ofO to 0.1 tg/ml (Fig. Two cultures of amniotic cells were grow[r] ...
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The Use of Neomycin in the Treatment of Methylmalonic Aciduria
... There was a marked drop in urinary excretion of methylmalonic acid and in the plasma level of this metabolite during both trials of neomycin therapy. These results are depicted graphical[r] ...
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Development and Validation of a GC-FID Method for Diagnosis of Methylmalonic Acidemia
... of methylmalonic acid in human urine ...of methylmalonic acid in thirty clinical urine samples were compared with those obtained by the gold standard GC-MS ...
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Neurocognitive Phenotype of Isolated Methylmalonic Acidemia
... Patient studies were conducted through National Institutes of Health study 04-HG-0127 ( “ Clinical and Basic Investigations of Methylmalonic Acid- emia and Related Disorders ” ; clinical- trials.gov identi ...
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Strength Developments and Deformation Characteristics of MMA-Modified Vinyl Ester Polymer Concrete
... to MMA contents are shown in ...at MMA contents of 0, ...for MMA contents of 0, ...as MMA contents increased. This shows that adding MMA is effective in reducing the coefficient of ...
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Low RCS Metamaterial Absorber and Extending Bandwidth Based on Electromagnetic Resonances
... in Figure 1 from the bottom to the top are denoted in sequence as 1st loop, 2nd loop and 3rd loop. As shown in Figure 3(b), 1st loop primarily couples with the electric field component, 2nd loop and 3rd loop response ...
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Comparison of methylation capacity among the people from the arsenic affected areas of West Bengal, India
... Chemicals Ltd. (Yamanashi, Japan). Trizma ® HCl was purchased from Sigma (St. Louis, MO, USA). Arsenocholine (AsC) and Standard Reference Material Toxic Metals in freeze-dried urine SRM-2670 (National Institute of ...
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THE INFLUENCE OF CURRANT MMA WELDING ON THE TENSILE STRENGHT OF JOINT
... The method MMA electrode coating is used, which consists of a metal core coated with com- pressed wrapper. Between the end of the elec - trode and the workpiece is produced electric arc. Arc ignition is a contact ...
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Failure of the Normal Ureagenic Response to Amino Acids in Organic Acid loaded Rats: PROPOSED MECHANISM FOR THE HYPERAMMONEMIA OF PROPIONIC AND METHYLMALONIC ACIDEMIA
... synthetase I, ornithine transcarbamylase, nor the activation constant (K A ) of carbamyl phosphate synthetase I for N-acetyl glutamate. Furthermore, rats injected with propionate showed no alteration of liver amino ...
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