Compounds > valerates
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valerates and Mitochondrial Diseases

valerates has been researched along with Mitochondrial Diseases in 5 studies

Research

Studies (5)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's2 (40.00)29.6817
2010's3 (60.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Antonetti, G; Boenzi, S; Carrozzo, R; Catesini, G; Diodato, D; Dionisi-Vici, C; Martinelli, D; Olivieri, G; Rizzo, C; Sacchetti, E; Semeraro, M1
Afroze, B; Ali, ZZ; Fatimah, M; Jafri, L; Jamil, A; Khan, AH; Majid, H; Yusufzai, N1
Amaral, AU; Fernandes, CG; Leipnitz, G; Schuck, PF; Seminotti, B; Wajner, M1
Almashanu, S; Baumgartner, ER; Baumgartner, MR; Dantas, MF; Fowler, B; Friebel, D; Gebhardt, B; Giunta, C; Hoffmann, GF; Suormala, T; Valle, D1
de Koning, T; Engelke, U; Huizing, M; Kluijtmans, LA; Loupatty, FJ; Morava, E; Rodenburg, RJ; Smeitink, JA; Wevers, R; Wortmann, S1

Other Studies

5 other study(ies) available for valerates and Mitochondrial Diseases

ArticleYear
The urinary organic acids profile in single large-scale mitochondrial DNA deletion disorders.
    Clinica chimica acta; international journal of clinical chemistry, 2018, Volume: 481

    Topics: 3-Hydroxybutyric Acid; Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Child; Child, Preschool; Congenital Bone Marrow Failure Syndromes; DNA, Mitochondrial; Fumarates; Glutarates; Humans; Hydroxybutyrates; Infant; Kearns-Sayre Syndrome; Lactic Acid; Lipid Metabolism, Inborn Errors; Mitochondrial Diseases; Muscular Diseases; Pyruvic Acid; Retrospective Studies; Valerates

2018
Diagnostic dilemma of patients with methylmalonic aciduria: Experience from a tertiary care centre in Pakistan.
    JPMA. The Journal of the Pakistan Medical Association, 2018, Volume: 68, Issue:4

    Topics: Alanine; Amino Acid Metabolism, Inborn Errors; Child, Preschool; Citrates; Cross-Sectional Studies; Female; Glycine; Humans; Infant; Lactic Acid; Male; Methionine; Mitochondrial Diseases; Pakistan; Tertiary Care Centers; Urinalysis; Valerates; Vitamin B 12 Deficiency

2018
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; Ketoglutarate Dehydrogenase Complex; Leucine; Maple Syrup Urine Disease; Membrane Potential, Mitochondrial; Mitochondrial Diseases; Mitochondrial Swelling; NADP; Oxygen Consumption; Prosencephalon; Rats; Rats, Wistar; Valerates

2010
Isolated 3-methylcrotonyl-CoA carboxylase deficiency: evidence for an allele-specific dominant negative effect and responsiveness to biotin therapy.
    American journal of human genetics, 2004, Volume: 75, Issue:5

    Topics: Alleles; Base Sequence; Biotin; Carbon-Carbon Ligases; DNA Mutational Analysis; Dose-Response Relationship, Drug; Fibroblasts; Gene Expression; Genetic Vectors; Germany; Glycine; Greece; Humans; Infant, Newborn; Male; Mitochondrial Diseases; Molecular Sequence Data; Mutation; Reverse Transcriptase Polymerase Chain Reaction; Sequence Analysis, DNA; Transfection; Valerates

2004
Association of 3-methylglutaconic aciduria with sensori-neural deafness, encephalopathy, and Leigh-like syndrome (MEGDEL association) in four patients with a disorder of the oxidative phosphorylation.
    Molecular genetics and metabolism, 2006, Volume: 88, Issue:1

    Topics: Adolescent; Brain Diseases, Metabolic; Child; Child, Preschool; Consanguinity; Fatal Outcome; Female; Glutarates; Hearing Loss, Sensorineural; Humans; Infant, Newborn; Leigh Disease; Male; Mitochondrial Diseases; Oxidative Phosphorylation; Syndrome; Valerates

2006