SMP0633620
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tRNA Charging
Pseudomonas aeruginosa
Matched Compounds: L-Leucine … L-Valine
Matched Proteins: Leucine-, isoleucine-, valine-, threonine-, and alanine-binding protein … leucine--tRNA ligase … valine--tRNA ligase
Matched Compound Synonyms: (2s)-alpha-leucine … (s)-(+)-leucine … (s)-leucine … Leucine … (2s)-a-leucine
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SMP0125675
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Isobutyryl-CoA Dehydrogenase Deficiency
Homo sapiens
Matched Description: error of metabolism (IEM) of valine metabolism. It is an autosomal recessive disorder that is caused … the metabolism of branched-chain amino acids, specifically valine. This enzyme is responsible for … the third step in the breakdown of valine and converts isobutyryl-CoA into methylacrylyl-CoA. Defects … in the IBD enzyme function lead to elevated levels of valine in blood and other biofluids … energy. Treatment may include the use of L-carnitine supplements, frequent meals, and a low-valine diet.
Matched Compounds: L-Valine … L-Leucine
Matched Compound Synonyms: (s)-valine … Valine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine
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SMP0691021
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Quorum Sensing: ComX Pheromone Biosynthesis (com Operon and srfA Operon Activation)
Escherichia coli
Matched Compounds: L-Leucine … L-Valine … D-Leucine
Matched Compound Synonyms: (2s)-alpha-leucine … (s)-(+)-leucine … (s)-leucine … Leucine … (2s)-a-leucine
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SMP0087234
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Valine, Leucine, and Isoleucine Degradation
Bos taurus
Matched Description: Valine, isoleuciine, and leucine are essential amino acids and are identified as the branched-chain … protein breakdown. In muscle, the final products of leucine, isoleucine, and valine catabolism can be … dehydrogenase (BCKD), yielding the three different CoA derivatives. Isovaleryl-CoA is produced from leucine … by these two reactions, alpha-methylbutyryl-CoA from isoleucine, and isobutyryl-CoA from valine … breakdown pathways then diverge. Leucine is ultimately converted into acetyl-CoA and acetoacetate
Matched Compounds: L-Valine … L-Leucine
Matched Compound Synonyms: (s)-valine … Valine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine
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SMP0000032
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Valine, Leucine, and Isoleucine Degradation
Homo sapiens
Matched Description: Valine, isoleuciine, and leucine are essential amino acids and are identified as the branched-chain … protein breakdown. In muscle, the final products of leucine, isoleucine, and valine catabolism can be … dehydrogenase (BCKD), yielding the three different CoA derivatives. Isovaleryl-CoA is produced from leucine … by these two reactions, alpha-methylbutyryl-CoA from isoleucine, and isobutyryl-CoA from valine … breakdown pathways then diverge. Leucine is ultimately converted into acetyl-CoA and acetoacetate
Matched Compounds: L-Leucine … L-Valine
Matched Compound Synonyms: (2s)-alpha-leucine … (s)-(+)-leucine … (s)-leucine … Leucine … (2s)-a-leucine
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SMP0000138
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3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency
Homo sapiens
Matched Description: 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency (3-Hydroxy-3-methylglutaric acidemia; Leucine
Matched Compounds: L-Leucine … L-Valine
Matched Compound Synonyms: (2s)-alpha-leucine … (s)-(+)-leucine … (s)-leucine … Leucine … (2s)-a-leucine
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SMP0000384
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Methylmalonate Semialdehyde Dehydrogenase Deficiency
Homo sapiens
Matched Compounds: L-Leucine … L-Valine
Matched Compound Synonyms: (2s)-alpha-leucine … (s)-(+)-leucine … (s)-leucine … Leucine … (2s)-a-leucine
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SMP0000522
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3-Hydroxyisobutyric Aciduria
Homo sapiens
Matched Description: metabolism (IEM) of valine metabolism. Only 12-13 patients have been identified with this condition. It … valine and pyrimidine catabolism. This protein catalyzes the irreversible oxidative decarboxylation
Matched Compounds: L-Leucine … L-Valine
Matched Compound Synonyms: (2s)-alpha-leucine … (s)-(+)-leucine … (s)-leucine … Leucine … (2s)-a-leucine
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SMP0120440
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3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency
Mus musculus
Matched Description: 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency (3-Hydroxy-3-methylglutaric acidemia; Leucine
Matched Compounds: L-Valine … L-Leucine
Matched Compound Synonyms: (s)-valine … Valine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine
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SMP0120806
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3-Hydroxyisobutyric Aciduria
Rattus norvegicus
Matched Description: metabolism (IEM) of valine metabolism. Only 12-13 patients have been identified with this condition. It … valine and pyrimidine catabolism. This protein catalyzes the irreversible oxidative decarboxylation
Matched Compounds: L-Valine … L-Leucine
Matched Compound Synonyms: (s)-valine … Valine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine
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