leucine and Mitochondrial Diseases

leucine has been researched along with Mitochondrial Diseases in 8 studies

Research

Studies (8)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's4 (50.00)29.6817
2010's4 (50.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Feng, Y; Geng, X; Jia, W; Jiang, Z; Li, F; Lu, H; Wang, C; Wei, X; Yan, J; Zhang, Y1
Costigan, D; Harty, S; King, MD; McCoy, B; Treacy, EP1
Cattepoel, S; Eckert, A; Kuehnle, K; Kulic, L; Nitsch, RM; Pagani, L; Rhein, V; Tracy, J; Wollmer, MA1
Amaral, AU; Fernandes, CG; Leipnitz, G; Schuck, PF; Seminotti, B; Wajner, M1
Bourke, B; Casey, JP; Conroy, J; Crushell, E; Ennis, S; Lynam-Lennon, N; Lynch, S; McDermott, M; McGettigan, P; O'Sullivan, J; Regan, R1
Brulé, H; Florentz, C; Frugier, M; Olszak, K; Przykorska, A; Sohm, B1
Gunay-Aygun, M1
Ahari, SE; Bahar, M; Banoei, MM; Houshmand, M; Kasraie, S; Moin, M; Panahi, MS; Shariati, P1

Reviews

1 review(s) available for leucine and Mitochondrial Diseases

ArticleYear
3-Methylglutaconic aciduria: a common biochemical marker in various syndromes with diverse clinical features.
    Molecular genetics and metabolism, 2005, Volume: 84, Issue:1

    Topics: Abnormalities, Multiple; Acetyl Coenzyme A; Amino Acid Metabolism, Inborn Errors; Glutarates; Humans; Hydro-Lyases; Leucine; Mitochondrial Diseases; Sterols

2005

Other Studies

7 other study(ies) available for leucine and Mitochondrial Diseases

ArticleYear
De Novo Mutation of m.3243A>G together with m.16093T>C Associated with Atypical Clinical Features in a Pedigree with MIDD Syndrome.
    Journal of diabetes research, 2019, Volume: 2019

    Topics: Adenosine Triphosphate; Adult; Aged; Aged, 80 and over; Deafness; Diabetes Mellitus, Type 2; DNA, Mitochondrial; Family Health; Female; Genetic Predisposition to Disease; Humans; Leucine; Leukocytes, Mononuclear; Magnetic Resonance Imaging; Male; Middle Aged; Mitochondrial Diseases; Mutation; Pedigree; Phenotype; Point Mutation; Reactive Oxygen Species; RNA, Transfer; Sequence Analysis, DNA

2019
Sensory-motor polyneuropathy occurring in variant maple syrup urine disease.
    Journal of inherited metabolic disease, 2008, Volume: 31 Suppl 2

    Topics: Adolescent; Biomarkers; Electromyography; Female; Humans; Leucine; Maple Syrup Urine Disease; Mitochondrial Diseases; Neural Conduction; Neurologic Examination; Peripheral Nerves; Polyneuropathies; Psychomotor Performance; Recurrence; Time Factors

2008
Combined expression of tau and the Harlequin mouse mutation leads to increased mitochondrial dysfunction, tau pathology and neurodegeneration.
    Neurobiology of aging, 2011, Volume: 32, Issue:10

    Topics: Adenosine Triphosphate; Age Factors; Animals; Apoptosis; Apoptosis Inducing Factor; Brain; Caspase 3; Electron Transport Complex I; Gene Expression Regulation; Genetic Predisposition to Disease; Humans; In Situ Nick-End Labeling; Leucine; Lipid Peroxidation; Male; Mice; Mice, Inbred C57BL; Mice, Mutant Strains; Mice, Transgenic; Mitochondria; Mitochondrial Diseases; Movement Disorders; Mutation; Nerve Degeneration; Nerve Tissue Proteins; Oxidative Stress; Proline; Proton Pumps; Silver Staining; Statistics, Nonparametric; tau Proteins; Tauopathies

2011
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
Identification of a mutation in LARS as a novel cause of infantile hepatopathy.
    Molecular genetics and metabolism, 2012, Volume: 106, Issue:3

    Topics: Adolescent; Adult; Amino Acyl-tRNA Synthetases; Base Sequence; Child; Child, Preschool; Cytoplasm; HEK293 Cells; Homozygote; Humans; Infant; Leucine; Liver Failure; Liver Failure, Acute; Mitochondria; Mitochondrial Diseases; Molecular Sequence Data; Mutation; Pedigree; Young Adult

2012
Towards understanding human mitochondrial leucine aminoacylation identity.
    Journal of molecular biology, 2003, May-16, Volume: 328, Issue:5

    Topics: Base Sequence; Genetic Variation; Humans; In Vitro Techniques; Leucine; Leucine-tRNA Ligase; Mitochondria; Mitochondrial Diseases; Molecular Sequence Data; Mutation; Nucleic Acid Conformation; Recombinant Proteins; RNA, Transfer, Leu; Solutions; Substrate Specificity

2003
Investigation of tRNA(Leu/Lys) and ATPase 6 genes mutations in Huntington's disease.
    Cellular and molecular neurobiology, 2008, Volume: 28, Issue:7

    Topics: Adenosine Triphosphatases; Amino Acid Substitution; DNA Damage; DNA Mutational Analysis; DNA, Mitochondrial; Genetic Predisposition to Disease; Genetic Testing; Humans; Huntington Disease; Leucine; Lysine; Mitochondrial Diseases; Mutation; RNA, Transfer

2008