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' returned 433 results

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
SMP0000237 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-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
SMP0000173 View Pathway	Metabolite beta-Ketothiolase Deficiency 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
SMP0000238 View Pathway	Metabolite Isovaleric Aciduria Homo sapiens Matched Description: of leucine. A defect in this enzyme causes accumulation of ammonia, ketone bodies, Isovaleryl/2 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
SMP0087234 View Pathway	Metabolite 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 BioPAX SVG SBGN SBML PWML All files (.zip) Metabolic
SMP0120662 View Pathway	Metabolite 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I Rattus norvegicus 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