Page last updated: 2024-10-17

methylmalonic acid and Acetonemia

methylmalonic acid has been researched along with Acetonemia in 12 studies

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.
methylmalonic acid : A dicarboxylic acid that is malonic acid in which one of the methylene hydrogens is substituted by a methyl group.

Research Excerpts

ExcerptRelevanceReference
"Methylmalonic acidemia is a heterogeneous inborn error of propionate metabolism."1.28Progressive renal insufficiency in methylmalonic acidemia. ( Friedman, AL; Molteni, KH; Oberley, TD; Wolff, JA, 1991)
"We suggested the diagnosis of type 4 renal tubular acidosis, subtype 2, i."1.28Type 4 renal tubular acidosis (subtype 2) in a patient with methylmalonic acidaemia. ( Abukawa, D; Aikawa, J; Hanamizu, H; Kikuchi, M; Narisawa, K; Ohura, T; Tada, K; Yunoki, H, 1990)

Research

Studies (12)

TimeframeStudies, this research(%)All Research%
pre-19906 (50.00)18.7374
1990's6 (50.00)18.2507
2000's0 (0.00)29.6817
2010's0 (0.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Giardini, O1
Marzetti, G1
Lubrano, R1
Laurenti, F1
Martino, F1
Mannarino, O1
D'Eufemia, P1
Ruberto, U1
Rosario, P1
Medina, JM1
Bibi, H1
Gelman-Kohan, Z1
Baumgartner, ER2
Rosenblatt, DS2
Chang, CC1
Hsiao, KJ1
Lee, YM1
Lin, CM1
Duran, M2
Bruinvis, L1
Ketting, D1
Wadman, SK1
Schreier, K1
Porath, U1
Bachmann, C1
Brechbühler, T1
Wick, H1
Leupold, D1
Molteni, KH1
Oberley, TD1
Wolff, JA1
Friedman, AL1
de Almeida, IT1
Silva, MF1
Portela, R1
Cabral, A1
Tasso, T1
Eusébio, F1
Silveira, C1
Shapira, SK1
Ledley, FD1
Levy, HL1
Ohura, T1
Kikuchi, M1
Abukawa, D1
Hanamizu, H1
Aikawa, J1
Narisawa, K1
Tada, K1
Yunoki, H1

Reviews

2 reviews available for methylmalonic acid and Acetonemia

ArticleYear
[Congenital metabolic acidosis in the postnatal period].
    Deutsche medizinische Wochenschrift (1946), 1978, Jun-02, Volume: 103, Issue:22

    Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Fructose-1,6-Diphosphatase Deficiency; Glutarates; G

1978
[Methylmalonic aciduria. Classification, diagnosis and therapy (author's transl)].
    Klinische Wochenschrift, 1977, Jan-15, Volume: 55, Issue:2

    Topics: Amino Acid Metabolism, Inborn Errors; Female; Humans; Infant, Newborn; Ketosis; Malonates; Methylmal

1977

Other Studies

10 other studies available for methylmalonic acid and Acetonemia

ArticleYear
[The neonatal acute form of methylmalonic acidemia. Report of 2 clinical cases].
    Minerva pediatrica, 1980, Sep-15, Volume: 32, Issue:17

    Topics: Acidosis; Acute Disease; Amino Acid Metabolism, Inborn Errors; Female; Humans; Hyperglycemia; Infant

1980
Stimulation of ketogenesis by propionate in isolated rat hepatocytes: an explanation for ketosis associated with propionic acidaemia and methylmalonic acidaemia?
    Journal of inherited metabolic disease, 1982, Volume: 5, Issue:1

    Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Animals; Carbon Dioxide; Cells, Cultured; Fatty Acid

1982
Transcobalamin II deficiency with methylmalonic aciduria in three sisters.
    Journal of inherited metabolic disease, 1999, Volume: 22, Issue:7

    Topics: Cells, Cultured; Female; Fibroblasts; Follow-Up Studies; Humans; Infant; Infant, Newborn; Ketosis; M

1999
Towards metabolic sink therapy for mut methylmalonic acidaemia: correction of methylmalonyl-CoA mutase deficiency in T lymphocytes from a mut methylmalonic acidaemia child by retroviral-mediated gene transfer.
    Journal of inherited metabolic disease, 1999, Volume: 22, Issue:7

    Topics: 3T3 Cells; Animals; Child; Feasibility Studies; Gene Transfer Techniques; Genetic Vectors; Humans; K

1999
Deranged isoleucine metabolism during ketotic attacks in patients with methylmalonic acidaemia.
    Journal of inherited metabolic disease, 1978, Volume: 1, Issue:3

    Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Child, Preschool; Chromatography, Gas; Female; Human

1978
Acute neonatal nonketotic hyperglycinemia: normal propionate and methylmalonate metabolism.
    Pediatric research, 1975, Volume: 9, Issue:7

    Topics: Acute Disease; Amino Acid Metabolism, Inborn Errors; Child; Child, Preschool; Coenzyme A; Female; Fi

1975
Progressive renal insufficiency in methylmalonic acidemia.
    Pediatric nephrology (Berlin, Germany), 1991, Volume: 5, Issue:3

    Topics: Adolescent; Creatinine; Dietary Proteins; Female; Glomerular Filtration Rate; Humans; Ketosis; Kidne

1991
Mild form of methylmalonic aciduria misdiagnosed as propionic acidaemia during a ketotic crisis.
    Journal of inherited metabolic disease, 1991, Volume: 14, Issue:2

    Topics: Amino Acid Metabolism, Inborn Errors; Diagnosis, Differential; Humans; Infant; Ketosis; Male; Methyl

1991
Ketoacidotic crisis as a presentation of mild ("benign") methylmalonic acidemia.
    The Journal of pediatrics, 1991, Volume: 119, Issue:1 Pt 1

    Topics: Acidosis; Amino Acids; Ammonia; Child, Preschool; Female; Fibroblasts; Humans; Ketones; Ketosis; Met

1991
Type 4 renal tubular acidosis (subtype 2) in a patient with methylmalonic acidaemia.
    European journal of pediatrics, 1990, Volume: 150, Issue:2

    Topics: Acidosis, Renal Tubular; Aldosterone; Biopsy; Humans; Infant; Ketosis; Kidney; Kidney Function Tests

1990