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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 (

Term:3-methylglutaconic aciduria type 1 pathway
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Accession:PW:0002274 term browser browse the term
Definition:A rare autosomal recessive condition resulting from alterations in leucine metabolism.
Synonyms:exact_synonym: 3-methylglutaconyl-CoA hydratase deficiency pathway;   3MG-CoA hydratase deficiency pathway;   MGA type I pathway;   MGA1 pathway;   type I 3-Methylglutaconic aciduria disease pathway;   type I 3-Methylglutaconic aciduria pathway
 related_synonym: SMP:00139

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3-methylglutaconic aciduria type 1 pathway term browser
Symbol Object Name Evidence Notes Source PubMed Reference(s) RGD Reference(s) Position
G Abat 4-aminobutyrate aminotransferase ISO SMPDB SMP:00139 NCBI chrNW_004936530:7,543,743...7,621,392 JBrowse link
G Acaa2 acetyl-CoA acyltransferase 2 ISO SMPDB SMP:00139 NCBI chrNW_004936497:13,242,644...13,269,007 JBrowse link
G Acad8 acyl-CoA dehydrogenase family member 8 ISO SMPDB SMP:00139 NCBI chrNW_004936684:1,572,386...1,591,231 JBrowse link
G Acadm acyl-CoA dehydrogenase medium chain ISO SMPDB SMP:00139 NCBI chrNW_004936571:2,761,254...2,797,045 JBrowse link
G Acads acyl-CoA dehydrogenase short chain ISO SMPDB SMP:00139 NCBI chrNW_004936668:257,214...272,089 JBrowse link
G Acadsb acyl-CoA dehydrogenase short/branched chain ISO SMPDB SMP:00139 NCBI chrNW_004936486:11,668,099...11,707,946 JBrowse link
G Acat1 acetyl-CoA acetyltransferase 1 ISO SMPDB SMP:00139 NCBI chrNW_004936551:594,647...626,016 JBrowse link
G Aldh2 aldehyde dehydrogenase 2 family member ISO SMPDB SMP:00139 NCBI chrNW_004936558:4,362,693...4,387,705 JBrowse link
G Aldh6a1 aldehyde dehydrogenase 6 family member A1 ISO SMPDB SMP:00139 NCBI chrNW_004936488:3,320,357...3,340,463 JBrowse link
G Aox1 aldehyde oxidase 1 ISO SMPDB SMP:00139 NCBI chrNW_004936825:263,725...332,424 JBrowse link
G Auh AU RNA binding methylglutaconyl-CoA hydratase ISO SMPDB SMP:00139 NCBI chrNW_004936796:160,335...302,458 JBrowse link
G Bcat1 branched chain amino acid transaminase 1 ISO SMPDB SMP:00139 NCBI chrNW_004936548:2,385,756...2,454,423 JBrowse link
G Bckdha branched chain keto acid dehydrogenase E1 subunit alpha ISO SMPDB SMP:00139 NCBI chrNW_004936661:3,799,998...3,817,560 JBrowse link
G Bckdhb branched chain keto acid dehydrogenase E1 subunit beta ISO SMPDB SMP:00139 NCBI chrNW_004936510:9,904,703...10,081,532 JBrowse link
G Dbt dihydrolipoamide branched chain transacylase E2 ISO SMPDB SMP:00139 NCBI chrNW_004936748:342,655...386,017 JBrowse link
G Dld dihydrolipoamide dehydrogenase ISO SMPDB SMP:00139 NCBI chrNW_004936479:16,247,230...16,271,597 JBrowse link
G Echs1 enoyl-CoA hydratase, short chain 1 ISO SMPDB SMP:00139 NCBI chrNW_004936486:19,488,619...19,496,583 JBrowse link
G Hibadh 3-hydroxyisobutyrate dehydrogenase ISO SMPDB SMP:00139 NCBI chrNW_004936478:4,118,752...4,219,908 JBrowse link
G Hibch 3-hydroxyisobutyryl-CoA hydrolase ISO SMPDB SMP:00139 NCBI chrNW_004936506:7,811,624...7,882,861 JBrowse link
G Hmgcl 3-hydroxy-3-methylglutaryl-CoA lyase ISO SMPDB SMP:00139 NCBI chrNW_004936474:8,955,216...8,971,078 JBrowse link
G Hmgcs2 3-hydroxy-3-methylglutaryl-CoA synthase 2 ISO SMPDB SMP:00139 NCBI chrNW_004936627:4,451,847...4,473,163 JBrowse link
G Hsd17b10 hydroxysteroid 17-beta dehydrogenase 10 ISO SMPDB SMP:00139 NCBI chrNW_004936751:399,743...402,200 JBrowse link
G Ivd isovaleryl-CoA dehydrogenase ISO SMPDB SMP:00139 NCBI chrNW_004936471:3,871,152...3,883,521 JBrowse link
G Mccc1 methylcrotonyl-CoA carboxylase subunit 1 ISO SMPDB SMP:00139 NCBI chrNW_004936566:409,558...468,898 JBrowse link
G Mccc2 methylcrotonyl-CoA carboxylase subunit 2 ISO SMPDB SMP:00139 NCBI chrNW_004936549:4,760,073...4,830,190 JBrowse link
G Mcee methylmalonyl-CoA epimerase ISO SMPDB SMP:00139 NCBI chrNW_004936483:971,148...990,634 JBrowse link
G Mmut methylmalonyl-CoA mutase ISO SMPDB SMP:00139 NCBI chrNW_004936476:11,208,251...11,241,648 JBrowse link
G Oxct1 3-oxoacid CoA-transferase 1 ISO SMPDB SMP:00139 NCBI chrNW_004936518:924,840...1,057,201 JBrowse link
G Pcca propionyl-CoA carboxylase subunit alpha ISO SMPDB SMP:00139 NCBI chrNW_004936472:10,167,387...10,501,824 JBrowse link
G Pccb propionyl-CoA carboxylase subunit beta ISO SMPDB SMP:00139 NCBI chrNW_004936529:9,106,525...9,166,311 JBrowse link

Term paths to the root
Path 1
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  pathway 3946
    disease pathway 686
      congenital disease pathway 506
        inborn genetic disease pathway 506
          inborn error of metabolism pathway 506
            3-methylglutaconic aciduria type 1 pathway 30
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