Methylmalonic Acid

A malonic acid derivative which is a vital intermediate in the metabolism of fat and protein. Abnormalities in methylmalonic acid metabolism lead to methylmalonic aciduria. This metabolic disease is attributed to a block in the enzymatic conversion of methylmalonyl CoA to succinyl CoA.

Also Known As:

Networked: 503 relevant articles (6 outcomes, 41 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Obeid, Rima: 11 articles (01/2022 - 07/2003)
2.	Herrmann, Wolfgang: 11 articles (02/2015 - 01/2002)
3.	Steiner, Jörg M: 6 articles (09/2021 - 01/2012)
4.	Allen, Robert H: 6 articles (01/2012 - 05/2002)
5.	Stabler, Sally P: 6 articles (01/2012 - 05/2002)
6.	Ueland, Per Magne: 5 articles (05/2022 - 03/2008)
7.	Suchodolski, Jan S: 5 articles (09/2021 - 01/2012)
8.	Green, Ralph: 5 articles (04/2019 - 08/2004)
9.	Ueland, Per M: 5 articles (01/2018 - 05/2005)
10.	Rosenblatt, David S: 5 articles (12/2016 - 09/2009)

Related Diseases

1.	Vitamin B 12 Deficiency (Vitamin B12 Deficiency) 01/01/2022 - "Vitamin B12 deficiency: correction of P-methylmalonic acid for estimated glomerular filtration rate to improve diagnostic value - a confirmatory study." 12/01/2015 - "Seventeen vegan or vegetarian adults (26-57 years of age) with a known vitamin B12 deficiency, as evidenced by a baseline serum methylmalonic acid (MMA) level above 270 nmol/L at screening, but who otherwise appeared healthy were enrolled in the study. " 01/01/2008 - "Recent studies have not investigated functional markers of vitamin B12 deficiency, such as methylmalonic acid. " 05/01/1987 - "If the high sensitivity and specificity of the gas chromatography-mass spectrometry assay for urinary methylmalonic acid is supported by other clinical studies, the methylmalonic acid assay may become the reference standard for the diagnosis of vitamin B12 deficiency." 01/01/1971 - "Clinical aspect of the studies of metabolic precursors of methylmalonic acid in vitamin B 12 deficiency."
2.	Methylmalonic acidemia 11/01/1981 - "Improved prenatal diagnosis of methylmalonic acidemia: mass fragmentography of methylmalonic acid in amniotic fluid and maternal urine." 11/01/2022 - "Methylmalonic acidemia (MMAemia) is characterized by accumulation of methylmalonic acid (MMA) in all body tissues. " 11/01/2022 - "Methylmalonic acidemia is a neurometabolic disorder biochemically characterized by the accumulation of methylmalonic acid (MMA) in different tissues, including the central nervous system (CNS). " 06/01/2019 - "Methylmalonic acidemia or aciduria (MMA) is an inborn error of metabolism that results in the accumulation of methylmalonic acid in blood with an increased excretion in urine. " 01/01/2019 - "Methylmalonic acidemia/aciduria (MMA) is a genetically heterogeneous group of inherited metabolic disorders biochemically characterized by the accumulation of methylmalonic acid. "
3.	Metabolic Diseases (Metabolic Disease) 05/01/1986 - "These studies support the efficacy of hydroxocobalamin therapy in this disease, suggest that methylmalonic acid may be the most appropriate metabolite to monitor for therapeutic response, and in importance of electrophysiologic studies in character in objectively monitoring the response to treatment metabolic disease." 11/30/2020 - "Methylmalonic acidemia is an inborn metabolic disease of propionate catabolism, biochemically characterized by accumulation of methylmalonic acid (MMA) to millimolar concentrations in tissues and body fluids. " 08/01/2003 - "Hyperammonemia is a common finding in children with methylmalonic acidemia, an inherited metabolic disease characterized by mental retardation, convulsions, and accumulation of methylmalonic acid (MMA). " 08/01/1982 - "Methylmalonic acidemia, an inherited metabolic disease affecting the catabolism of propionic acid, is manifested by persistent metabolic acidosis, urinary excretion of large amounts of methylmalonic acid, and occasionally by hypoglycemia. " 01/01/1991 - "Organic acids have been determined in aqueous humour and plasma by gas chromatography-mass spectrometry in 38 cases of infant death and 4 cases of inherited metabolic disease: one had a complex fatty-acid oxidation disorder with a large urinary excretion of adipic acid, the others had a disorder of propionate catabolism with a large urinary excretion of methylmalonic acid. "
4.	Chronic Kidney Failure (Chronic Renal Failure) 08/01/2007 - "Chronic renal failure (CRF) was found most frequently in mut0 (61%) and cblB patients (66%), and was predicted by the urinary excretion of methylmalonic acid (MMA) before CRF. " 03/01/2005 - "However, it is not yet known whether methylmalonic acid is involved in the induction of other organ manifestations in this disease, such as chronic renal failure." 06/01/1992 - "During a 3 month period we measured serum methylmalonic acid concentrations monthly in 37 patients, all on chronic haemodialysis because of end-stage kidney disease. " 01/01/2013 - "Serum methylmalonic acid and holotranscobalamin-II as markers for vitamin B12 deficiency in end-stage renal disease patients." 01/01/2013 - "Serum vitamin B12 and methylmalonic acid (MMA) levels were drawn from ESRD patients prior to hemodialysis. "
5.	Maple Syrup Urine Disease 12/01/1979 - "Osmotic diuresis increases the 45 ml/min. However it is of little elimination of methylmalonic acid because the renal clearance is between 15 and 45 ml/min. However it is of little value in maple syrup urine disease, propionic acidaemia or isovaleric acidaemia because the renal clearance of the toxic metabolites is so low. " 01/01/2014 - "This retrospective study included patients with urea cycle disorders, maple syrup urine disease, and methylmalonic acidemia who received IHD or non-IHD RRT at our hospital between 2001 and 2012 to remove ammonia, leucine, or methylmalonic acid. "

Related Drugs and Biologics

1.	Vitamin B 12 (Cyanocobalamin)
2.	Homocysteine
3.	Folic Acid (Vitamin M)
4.	Biomarkers (Surrogate Marker)
5.	Hydroxocobalamin
6.	Creatinine
7.	Indicators and Reagents (Reagents)
8.	Vitamins
9.	Metformin (Glucophage)
10.	Cystathionine

Related Therapies and Procedures

1.	Therapeutics
2.	Injections
3.	Renal Dialysis (Hemodialysis)
4.	Aftercare (After-Treatment)
5.	Liver Transplantation