PathBank

Filter by Species:

Filter by Pathway Type:

Select Pathway Sub-Category:

Select Pathway Sub-Category:

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

SMP0121281 View Pathway	Metabolite 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 BioPAX SVG SBGN SBML PWML All files (.zip) Metabolic
SMP0000820 View Pathway	Metabolite tRNA Charging Escherichia coli 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) Metabolic
SMP0125661 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-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
SMP0125675 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-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
SMP0120808 View Pathway	Metabolite Isovaleric Acidemia Rattus norvegicus Matched Description: (IEM) of leucine metabolism. It is an autosomal recessive disorder that is caused by a deficiency of … the conjugate. A diet that is also restricted in leucine consumption is also useful in treating the disorder. 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
SMP0120516 View Pathway	Metabolite Maple Syrup Urine Disease Mus musculus 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-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
SMP0120442 View Pathway	Metabolite 3-Methylglutaconic Aciduria Type I Mus musculus 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
SMP0120589 View Pathway	Metabolite Isovaleric Acidemia Mus musculus Matched Description: (IEM) of leucine metabolism. It is an autosomal recessive disorder that is caused by a deficiency of … the conjugate. A diet that is also restricted in leucine consumption is also useful in treating the disorder. 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
SMP0125658 View Pathway	Metabolite 2-Methyl-3-hydroxybutyryl-CoA 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
SMP0125670 View Pathway	Metabolite Methylmalonic Aciduria 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