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humans (116) 116
isovaleryl-coa dehydrogenase (109) 109
oxidoreductases acting on ch-ch group donors (70) 70
index medicus (58) 58
male (50) 50
female (45) 45
isovaleryl-coa dehydrogenase - deficiency (44) 44
biochemistry & molecular biology (42) 42
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pentanoic acids - blood (35) 35
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isovaleryl-coa dehydrogenase - genetics (30) 30
oxidoreductases - genetics (29) 29
genetics & heredity (28) 28
molecular sequence data (28) 28
amino acid sequence (27) 27
infant (27) 27
metabolism (26) 26
isovaleryl-coa dehydrogenase - metabolism (25) 25
child (24) 24
isovaleryl-coa (24) 24
amino acid metabolism, inborn errors - diagnosis (23) 23
amino acid metabolism, inborn errors - genetics (23) 23
identification (23) 23
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oxidoreductases - metabolism (23) 23
pediatrics (21) 21
rats (21) 21
acyl-coa dehydrogenase (20) 20
analysis (20) 20
amino acids (19) 19
base sequence (18) 18
biosynthesis (17) 17
enzymes (17) 17
mass spectrometry (17) 17
physiological aspects (17) 17
plant sciences (17) 17
substrate specificity (17) 17
tandem mass spectrometry (17) 17
acyl coenzyme a - metabolism (16) 16
medicine, research & experimental (16) 16
rat-liver mitochondria (16) 16
deficiency (15) 15
microbiology (15) 15
purification (15) 15
research (15) 15
tandem mass-spectrometry (15) 15
amino acid metabolism, inborn errors - metabolism (14) 14
carnitine - analogs & derivatives (14) 14
carnitine - blood (14) 14
genes (14) 14
genetic aspects (14) 14
kinetics (14) 14
molecular-cloning (14) 14
isovaleryl-coa-dehydrogenase (13) 13
mitochondria, liver - enzymology (13) 13
models, molecular (13) 13
phenotype (13) 13
proteins (13) 13
amino acid metabolism, inborn errors - blood (12) 12
amino acid metabolism, inborn errors - enzymology (12) 12
dehydrogenases (12) 12
fibroblasts (12) 12
leucine (12) 12
mitochondria (12) 12
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valerates - blood (12) 12
acidemia (11) 11
cloning, molecular (11) 11
glycine - analogs & derivatives (11) 11
isovaleryl-coa dehydrogenase - blood (11) 11
nucleotide-sequence (11) 11
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cells, cultured (10) 10
electron-transfer flavoprotein (10) 10
expression (10) 10
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pentanoic acids - metabolism (10) 10
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branched chain amino acids (9) 9
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coenzyme-a dehydrogenase (9) 9
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endocrinology & metabolism (9) 9
fatty acids (9) 9
fibroblasts - enzymology (9) 9
medium-chain (9) 9
mutation - genetics (9) 9
sequence homology, amino acid (9) 9
acyl-coa dehydrogenases (8) 8
beta-oxidation (8) 8
biochemistry (8) 8
carnitine (8) 8
gas chromatography-mass spectrometry (8) 8
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1. Full Text Deuterium incorporation experiments from (3R)- and (3S)-[3-2H]leucine into characteristic isoprenoidal lipid-core of halophilic archaea suggests the involvement of isovaleryl-CoA dehydrogenase
by Yamauchi, Noriaki and Tanoue, Ryo
Bioscience, Biotechnology, and Biochemistry, ISSN 0916-8451, 11/2017, Volume 81, Issue 11, pp. 2062 - 2070
The stereochemical reaction course for the two C-3 hydrogens of leucine to produce a characteristic isoprenoidal lipid in halophilic archaea was observed using... 
leucine | isovaleryl-CoA | halophilic archaea | deuterium-labeling
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2. Full Text The Regulation of Essential Amino Acid Synthesis and Accumulation in Plants
by Galili, Gad and Amir, Rachel and Fernie, Alisdair R
Annual Review of Plant Biology, ISSN 1543-5008, 4/2016, Volume 67, Issue 1, pp. 153 - 178
Although amino acids are critical for all forms of life, only proteogenic amino acids that humans and animals cannot synthesize de novo and therefore must... 
cellular energy | nutrition | metabolism | Nutrition | Metabolism | Cellular energy | BACTERIAL DIHYDRODIPICOLINATE SYNTHASE | PLANT SCIENCES | CYSTATHIONINE-GAMMA-SYNTHASE | ADENOSYL-L-METHIONINE | ISOVALERYL-COA-DEHYDROGENASE | INCREASED LYSINE SYNTHESIS | ELECTRON-TRANSFER FLAVOPROTEIN | 3-DEOXY-D-ARABINO-HEPTULOSONATE 7-PHOSPHATE SYNTHASE | TRANSGENIC POTATO PLANTS | DESENSITIZED ASPARTATE KINASE | RESISTANT ANTHRANILATE SYNTHASE | Plant Proteins - genetics | Genetic Engineering | Crops, Agricultural - enzymology | Genes, Plant | Crops, Agricultural - genetics | Plant Proteins - metabolism | Amino Acids, Essential - biosynthesis | Amino Acids, Essential - metabolism | Crops, Agricultural - metabolism | Amino acids | Cellular signal transduction | Synthesis | Observations
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3. Full Text Identification of the 2-Hydroxyglutarate and Isovaleryl-CoA Dehydrogenases as Alternative Electron Donors Linking Lysine Catabolism to the Electron Transport Chain of Arabidopsis Mitochondria
by Wagner L. Araújo and Kimitsune Ishizaki and Adriano Nunes-Nesi and Tony R. Larson and Takayuki Tohge and Ina Krahnert and Sandra Witt and Toshihiro Obata and Nicolas Schauer and Ian A. Graham and Christopher J. Leaver and Alisdair R. Fernie
The Plant Cell, ISSN 1040-4651, 5/2010, Volume 22, Issue 5, pp. 1549 - 1563
The process of dark-induced senescence in plants is relatively poorly understood, but a functional electron-transfer flavoprotein/electron-transfer... 
Enzymes | Leaves | Phenotypes | Dehydrogenases | Amino acids | Catabolism | Plants | Amino acid metabolism | Chlorophylls | Plant cells | FLAVOPROTEIN-UBIQUINONE OXIDOREDUCTASE | BETA-OXIDATION | BIOCHEMISTRY & MOLECULAR BIOLOGY | EXTENDED DARKNESS | PLANT SCIENCES | CELL BIOLOGY | LEAF SENESCENCE | PIPECOLIC ACID | GAS-CHROMATOGRAPHY | METABOLISM | AMINO-ACIDS | PLANTS | CHLOROPHYLL BREAKDOWN | Darkness | Leucine - metabolism | Mitochondria - enzymology | Electron Transport | Arabidopsis - enzymology | Gas Chromatography-Mass Spectrometry | Carbohydrate Metabolism | Metabolome | Alcohol Oxidoreductases - metabolism | Mutation - genetics | Isovaleryl-CoA Dehydrogenase - metabolism | Mutagenesis, Insertional - genetics | Phytol - metabolism | Phenotype | DNA, Bacterial - genetics | Plant Leaves - metabolism | Models, Biological | Isotope Labeling | Lysine - metabolism | Acyl Coenzyme A - metabolism | Electrons | Arabidopsis thaliana | Plant mitochondria | Growth | Genetic aspects | Properties | Electron transport | Observations | Glutamine
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4. Full Text Respiratory electron transfer pathways in plant mitochondria
by Schertl, Peter and Braun, Hans-Peter
Frontiers in Plant Science, ISSN 1664-462X, 04/2014, Volume 5, p. 163
The respiratory electron transport chain (ETC) couples electron transfer from organic substrates onto molecular oxygen with proton translocation across the... 
Electron transport chain | Respiratory supercomplex | Plant mitochondria | Alternative oxidase | Dehydrogenase | THALIANA PYRUVATE-DEHYDROGENASE | electron transport chain | GAMMA-LACTONE DEHYDROGENASE | GLYCINE DECARBOXYLASE COMPLEX | plant mitochondria | respiratory supercomplex | dehydrogenase | AMINO-ACID CATABOLISM | ALPHA-KETOGLUTARATE DEHYDROGENASE | DEPENDENT ISOCITRATE DEHYDROGENASE | CYTOCHROME-C REDUCTASE | PLANT SCIENCES | ISOVALERYL-COA-DEHYDROGENASE | NAD(H)-DEPENDENT GLUTAMATE-DEHYDROGENASE | SUCCINIC-SEMIALDEHYDE DEHYDROGENASE | alternative oxidase | Alternative Oxidase
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5. Full Text A novel missense mutation of Isovaleryl-CoA dehydrogenase gene associated with chronic intermittent Isovaleric acidemia in a Bangladeshi patient
by Sarker, Suprovath Kumar and Islam, Md Tarikul and Hasib, Saad Hassan and Sultana, Nusrat and Sultana, Rosy and Hossain, Shekh Rezwan and Biswas, Aparna and Bhuyan, Golam Sarower and Begum, Mst. Noorjahan and Konica, Fatema Ahmed and Qadri, Syed Saleheen and Qadri, Firdausi and Qadri, Syeda Kashfi and Saha, Narayan and Mannoor, Kaiissar
Meta Gene, ISSN 2214-5400, 06/2019, Volume 20, p. 100557
Isovaleric acidemia (IVA) is an organic acidemia characterized by the deficiency of isovaleryl-CoA dehydrogenase (IVD) enzyme, of the third step in the leucine... 
Isovaleryl-CoA dehydrogenase | FAD | Case report | Bangladesh | Organic acidemia | Isovaleric acidemia
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6. Full Text Genotype-phenotype correlation in patients with isovaleric acidaemia: Comparative structural modelling and computational analysis of novel variants
by Zaki, Osama K and George Priya Doss, C and Ali, Salsabil A and Murad, Ghadeer G and Elashi, Shaima A and Ebnou, Maryam S.A and Thirumal Kumar, D and Khalifa, Ola and Gamal, Radwa and El Abd, Heba S.A and Nasr, Bilal N and Zayed, Hatem
Human Molecular Genetics, ISSN 0964-6906, 08/2017, Volume 26, Issue 16, pp. 3105 - 3115
Isovaleric acidaemia (IVA) is an autosomal recessive inborn error of leucine metabolism. It is caused by a deficiency in the mitochondrial isovaleryl-CoA... 
IVD GENE | DELETERIOUS NSSNPS | BIOCHEMISTRY & MOLECULAR BIOLOGY | SEQUENCE | GENETICS & HEREDITY | MUTATIONS | SPECTRUM | DEHYDROGENASE | Introns - genetics | Humans | Carnitine - blood | Child, Preschool | Genetic Association Studies - methods | Exons - genetics | Infant | Male | Isovaleryl-CoA Dehydrogenase - genetics | Isovaleryl-CoA Dehydrogenase - metabolism | Amino Acid Metabolism, Inborn Errors - genetics | Isovaleryl-CoA Dehydrogenase - deficiency | Base Sequence | RNA Splicing - genetics | Female | Polymorphism, Genetic - genetics | Mutation | Child
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7. Full Text Impact of Branched-Chain Amino Acid Catabolism on Fatty Acid and Alkene Biosynthesis in Micrococcus luteus
by Surger, Maximilian J and Angelov, Angel and Stier, Philipp and Übelacker, Maria and Liebl, Wolfgang
Frontiers in microbiology, ISSN 1664-302X, 2018, Volume 9, p. 374
naturally produces alkenes, unsaturated aliphatic hydrocarbons, and represents a promising host to produce hydrocarbons as constituents of biofuels and... 
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8. Full Text Branched‐chain amino acid metabolism in higher plants
by Binder, Stefan and Knill, Tanja and Schuster, Joachim
Physiologia Plantarum, ISSN 0031-9317, 01/2007, Volume 129, Issue 1, pp. 68 - 78
Valine, leucine and isoleucine contain short branched carbohydrate residues responsible for their classification as branched‐chain amino acids (BCAA). Among... 
ISOVALERYL-COA-DEHYDROGENASE | ARABIDOPSIS-THALIANA | EXPRESSION ANALYSIS | 3-METHYLCROTONYL-COENZYME-A CARBOXYLASE | CRYSTAL-STRUCTURE | BIOSYNTHESIS | GENE FAMILY | MOLECULAR-CLONING | LEUCINE SYNTHESIS | ACYL-COA | PLANT SCIENCES
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9. Full Text MoIVD-Mediated Leucine Catabolism Is Required for Vegetative Growth, Conidiation and Full Virulence of the Rice Blast Fungus Magnaporthe oryzae
by Li, Y and Zheng, XX and Zhu, MH and Chen, MT and Zhang, SN and He, FY and Chen, XM and Lv, JR and Pei, MT and Zhang, Y and Zhang, YH and Wang, WZ and Zhang, J and Wang, M