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Pathways
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PATHWAY ONTOLOGY - ANNOTATIONS

The Pathway Ontology (PW), is currently being developed at the Rat Genome Database. For more information about this vocabulary, please see Petri et al. The rat genome database pathway portal. Database (Oxford). 2011 Apr 8;2011:bar010. Print 2011 or contact us (http://rgd.mcw.edu/contact/index.shtml).
Term:isovaleric acidemia pathway
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Accession:PW:0001874 term browser browse the term
Definition:A rare autosomal condition resulting from an impaired amino acid metabolism.
Synonyms:exact_synonym: isovaleric acid CoA dehydrogenase deficiency pathway;   isovaleric acidemia disease pathway;   isovaleric aciduria pathway;   isovaleryl-CoA dehydrogenase deficiency pathway
 related_synonym: SMP:00238;   SMP:00524;   isovaleric aciduria disease pathway


show annotations for term's descendants           Sort by:
isovaleric acidemia pathway term browser
Symbol Object Name Evidence Notes Source PubMed Reference(s) RGD Reference(s) Position
G ABAT 4-aminobutyrate aminotransferase ISO SMPDB SMP:00238 SMP:00524 NCBI chr 6:33,385,779...33,471,348
Ensembl chr 6:33,376,996...33,471,326 		JBrowse link
G ACAA2 acetyl-CoA acyltransferase 2 ISO SMPDB SMP:00238 SMP:00524 NCBI chr 7:79,028,636...79,057,720 JBrowse link
G ACAD8 acyl-CoA dehydrogenase family member 8 ISO SMPDB SMP:00238 SMP:00524 NCBI chr 5:974,673...992,143
Ensembl chr 5:976,008...992,330 		JBrowse link
G ACADM acyl-CoA dehydrogenase medium chain ISO SMPDB SMP:00238 SMP:00524 NCBI chr 6:70,852,223...70,881,979
Ensembl chr 6:70,853,061...70,878,668 		JBrowse link
G ACADS acyl-CoA dehydrogenase short chain ISO SMPDB SMP:00238 SMP:00524 NCBI chr26:16,572,936...16,584,760
Ensembl chr26:16,572,947...16,584,194 		JBrowse link
G ACADSB acyl-CoA dehydrogenase short/branched chain ISO SMPDB SMP:00238 SMP:00524 NCBI chr28:32,563,697...32,604,006
Ensembl chr28:32,563,671...32,601,063 		JBrowse link
G ACAT1 acetyl-CoA acetyltransferase 1 ISO SMPDB SMP:00238 SMP:00524 NCBI chr 5:24,361,301...24,379,807
Ensembl chr 5:24,361,301...24,379,812 		JBrowse link
G ALDH2 aldehyde dehydrogenase 2 family member ISO SMPDB SMP:00238 SMP:00524 NCBI chr26:9,319,109...9,361,021 JBrowse link
G ALDH6A1 aldehyde dehydrogenase 6 family member A1 ISO SMPDB SMP:00238 SMP:00524 NCBI chr 8:47,280,872...47,304,460
Ensembl chr 8:47,282,235...47,304,283 		JBrowse link
G AOX2 aldehyde oxidase 2 ISO SMPDB SMP:00238 SMP:00524 NCBI chr37:9,912,731...9,992,322
Ensembl chr37:9,913,675...9,992,319 		JBrowse link
G AUH AU RNA binding methylglutaconyl-CoA hydratase ISO SMPDB SMP:00238 SMP:00524 NCBI chr 1:95,547,822...95,707,654
Ensembl chr 1:95,547,843...95,707,471 		JBrowse link
G BCAT1 branched chain amino acid transaminase 1 ISO SMPDB SMP:00238 SMP:00524 NCBI chr27:22,536,297...22,643,350
Ensembl chr27:22,535,254...22,636,959 		JBrowse link
G BCKDHA branched chain keto acid dehydrogenase E1 subunit alpha ISO SMPDB SMP:00238 SMP:00524 NCBI chr 1:112,579,345...112,599,163 JBrowse link
G BCKDHB branched chain keto acid dehydrogenase E1 subunit beta ISO SMPDB SMP:00238 SMP:00524 NCBI chr12:41,009,798...41,224,802
Ensembl chr12:41,009,730...41,224,802 		JBrowse link
G DBT dihydrolipoamide branched chain transacylase E2 ISO SMPDB SMP:00238 SMP:00524 NCBI chr 6:49,753,233...49,805,493
Ensembl chr 6:49,753,323...49,805,882 		JBrowse link
G DLD dihydrolipoamide dehydrogenase ISO SMPDB SMP:00238 SMP:00524 NCBI chr18:12,703,340...12,734,408
Ensembl chr18:12,703,341...12,734,479 		JBrowse link
G ECHS1 enoyl-CoA hydratase, short chain 1 ISO SMPDB SMP:00238 SMP:00524 NCBI chr28:40,939,856...40,947,694
Ensembl chr28:40,939,961...40,946,167 		JBrowse link
G HIBADH 3-hydroxyisobutyrate dehydrogenase ISO SMPDB SMP:00238 SMP:00524 NCBI chr14:40,672,294...40,780,608
Ensembl chr14:40,673,008...40,780,516 		JBrowse link
G HIBCH 3-hydroxyisobutyryl-CoA hydrolase ISO SMPDB SMP:00238 SMP:00524 NCBI chr37:841,385...943,972
Ensembl chr37:841,414...943,941 		JBrowse link
G HMGCL 3-hydroxy-3-methylglutaryl-CoA lyase ISO SMPDB SMP:00238 SMP:00524 NCBI chr 2:75,688,736...75,703,095
Ensembl chr 2:75,688,827...75,702,965 		JBrowse link
G HMGCS2 3-hydroxy-3-methylglutaryl-CoA synthase 2 ISO SMPDB SMP:00238 SMP:00524 NCBI chr17:56,679,942...56,698,521
Ensembl chr17:56,680,301...56,698,419 		JBrowse link
G HSD17B10 hydroxysteroid 17-beta dehydrogenase 10 ISO SMPDB SMP:00238 SMP:00524 NCBI chr  X:45,165,272...45,167,683
Ensembl chr  X:45,165,272...45,167,574 		JBrowse link
G IVD isovaleryl-CoA dehydrogenase ISO SMPDB SMP:00238 SMP:00524 NCBI chr30:7,584,122...7,594,599
Ensembl chr30:7,584,086...7,594,150 		JBrowse link
G MCCC1 methylcrotonyl-CoA carboxylase subunit 1 ISO SMPDB SMP:00238 SMP:00524 NCBI chr34:16,022,493...16,084,346
Ensembl chr34:16,022,499...16,084,387 		JBrowse link
G MCCC2 methylcrotonyl-CoA carboxylase subunit 2 ISO SMPDB SMP:00238 SMP:00524 NCBI chr 2:54,751,703...54,825,133
Ensembl chr 2:54,751,698...54,823,273 		JBrowse link
G MCEE methylmalonyl-CoA epimerase ISO SMPDB SMP:00238 SMP:00524 NCBI chr 3:38,080,570...38,107,832
Ensembl chr 3:38,080,611...38,107,830 		JBrowse link
G MMUT methylmalonyl-CoA mutase ISO SMPDB SMP:00238 SMP:00524 NCBI chr12:17,306,074...17,339,921
Ensembl chr12:17,307,522...17,339,897 		JBrowse link
G OXCT1 3-oxoacid CoA-transferase 1 ISO SMPDB SMP:00238 SMP:00524 NCBI chr 4:67,709,846...67,841,737
Ensembl chr 4:67,527,531...67,841,518 		JBrowse link
G PCCA propionyl-CoA carboxylase subunit alpha ISO SMPDB SMP:00238 SMP:00524 NCBI chr22:49,965,134...50,428,025
Ensembl chr22:50,023,369...50,428,027 		JBrowse link
G PCCB propionyl-CoA carboxylase subunit beta ISO SMPDB SMP:00238 SMP:00524 NCBI chr23:32,689,732...32,782,204
Ensembl chr23:32,687,839...32,836,366 		JBrowse link
Term paths to the root
Path 1
Term Annotations click to browse term
  pathway 4052
    disease pathway 717
      congenital disease pathway 519
        inborn genetic disease pathway 519
          inborn error of metabolism pathway 519
            inborn error of amino acid metabolism pathway 229
              isovaleric acidemia pathway 30
paths to the root