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Search for 'valine AND leucine' in 'all pathways' in 'all' returned 116 results

SMP0120745 View Pathway	Metabolite Methylmalonic Aciduria Rattus norvegicus Matched Compounds: L-Valine … L-Leucine Matched Compound Synonyms: (s)-valine … Valine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine BioPAX SVG SBGN SBML PWML All files (.zip) Disease
SMP0120807 View Pathway	Metabolite Isobutyryl-CoA Dehydrogenase Deficiency Rattus norvegicus 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 BioPAX SVG SBGN SBML PWML All files (.zip) Disease
SMP0120441 View Pathway	Metabolite 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I Mus musculus Matched Description: constitute the breakdown of leucine. Mutations in the aforementioned genes leads to a reduction in the … breakdown leucine properly. Consequently, this leads to the build up of toxic byproducts which are not … processed as the breakdown of leucine is left incomplete. If these toxic byproducts manifest themselves Matched Compounds: L-Valine … L-Leucine Matched Compound Synonyms: (s)-valine … Valine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine BioPAX SVG SBGN SBML PWML All files (.zip) Disease
SMP0120588 View Pathway	Metabolite Isobutyryl-CoA Dehydrogenase Deficiency Mus musculus 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 BioPAX SVG SBGN SBML PWML All files (.zip) Disease
SMP0000200 View Pathway	Metabolite Methylmalonic Aciduria Homo sapiens Matched Compounds: L-Leucine … L-Valine Matched Compound Synonyms: (2s)-alpha-leucine … (s)-(+)-leucine … (s)-leucine … Leucine … (2s)-a-leucine BioPAX SVG SBGN SBML PWML All files (.zip) Disease
SMP0000523 View Pathway	Metabolite 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-Leucine … L-Valine Matched Compound Synonyms: (2s)-alpha-leucine … (s)-(+)-leucine … (s)-leucine … Leucine … (2s)-a-leucine BioPAX SVG SBGN SBML PWML All files (.zip) Disease
SMP0000199 View Pathway	Metabolite Maple Syrup Urine Disease Homo sapiens Matched Description: for a protein which is vital in the breakdown of amino acids, specifically the amino acids leucine … , isoleucine and valine. This disorder is characterized by a large accumulation of these amino acids Matched Compounds: L-Leucine … L-Valine Matched Compound Synonyms: (2s)-alpha-leucine … (s)-(+)-leucine … (s)-leucine … Leucine … (2s)-a-leucine BioPAX SVG SBGN SBML PWML All files (.zip) Disease
SMP0125662 View Pathway	Metabolite 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I Homo sapiens Matched Description: constitute the breakdown of leucine. Mutations in the aforementioned genes leads to a reduction in the … breakdown leucine properly. Consequently, this leads to the build up of toxic byproducts which are not … processed as the breakdown of leucine is left incomplete. If these toxic byproducts manifest themselves Matched Compounds: L-Valine … L-Leucine Matched Compound Synonyms: (s)-valine … Valine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine BioPAX SVG SBGN SBML PWML All files (.zip) Disease
SMP0125669 View Pathway	Metabolite Methylmalonate Semialdehyde Dehydrogenase Deficiency Homo sapiens Matched Compounds: L-Valine … L-Leucine Matched Compound Synonyms: (s)-valine … Valine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine BioPAX SVG SBGN SBML PWML All files (.zip) Disease
SMP0125657 View Pathway	Metabolite beta-Ketothiolase Deficiency Homo sapiens Matched Compounds: L-Valine … L-Leucine Matched Compound Synonyms: (s)-valine … Valine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine BioPAX SVG SBGN SBML PWML All files (.zip) Disease