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2-keto-4-methylvalerate and Mitochondrial Diseases

2-keto-4-methylvalerate has been researched along with Mitochondrial Diseases in 2 studies

alpha-ketoisocaproic acid: RN given refers to parent cpd
4-methyl-2-oxopentanoate : A 2-oxo monocarboxylic acid anion that is the conjugate base of 4-methyl-2-oxopentanoic acid.
4-methyl-2-oxopentanoic acid : A 2-oxo monocarboxylic acid that is pentanoic acid (valeric acid) substituted with a keto group at C-2 and a methyl group at C-4. A metabolite that has been found to accumulate in maple syrup urine disease.

Mitochondrial Diseases: Diseases caused by abnormal function of the MITOCHONDRIA. They may be caused by mutations, acquired or inherited, in mitochondrial DNA or in nuclear genes that code for mitochondrial components. They may also be the result of acquired mitochondria dysfunction due to adverse effects of drugs, infections, or other environmental causes.

Research

Studies (2)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's1 (50.00)29.6817
2010's1 (50.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Amaral, AU1
Leipnitz, G1
Fernandes, CG1
Seminotti, B1
Schuck, PF1
Wajner, M1
Parra, D1
González, A1
Mugueta, C1
Martínez, A1
Monreal, I1

Reviews

1 review available for 2-keto-4-methylvalerate and Mitochondrial Diseases

ArticleYear
Laboratory approach to mitochondrial diseases.
    Journal of physiology and biochemistry, 2001, Volume: 57, Issue:3

    Topics: Breath Tests; DNA, Mitochondrial; Humans; Keto Acids; MERRF Syndrome; Mitochondria; Mitochondrial Di

2001

Other Studies

1 other study available for 2-keto-4-methylvalerate and Mitochondrial Diseases

ArticleYear
Alpha-ketoisocaproic acid and leucine provoke mitochondrial bioenergetic dysfunction in rat brain.
    Brain research, 2010, Apr-09, Volume: 1324

    Topics: Animals; Brain; Central Nervous System Agents; Electron Transport; Homeostasis; Keto Acids; Ketoglut

2010