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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: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
Ensembl chrNW_004936530:7,543,682...7,622,667
JBrowse link
G Acaa2 acetyl-CoA acyltransferase 2 ISO SMPDB SMP:00139 NCBI chrNW_004936497:13,242,644...13,269,007
Ensembl chrNW_004936497:13,242,644...13,269,532
JBrowse link
G Acad8 acyl-CoA dehydrogenase family member 8 ISO SMPDB SMP:00139 NCBI chrNW_004936684:1,572,386...1,591,231
Ensembl chrNW_004936684:1,575,955...1,591,212
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
Ensembl chrNW_004936668:254,595...272,118
JBrowse link
G Acadsb acyl-CoA dehydrogenase short/branched chain ISO SMPDB SMP:00139 NCBI chrNW_004936486:11,668,099...11,707,946
Ensembl chrNW_004936486:11,668,099...11,711,786
JBrowse link
G Acat1 acetyl-CoA acetyltransferase 1 ISO SMPDB SMP:00139 NCBI chrNW_004936551:594,647...626,016
Ensembl chrNW_004936551:591,695...626,056
JBrowse link
G Aldh2 aldehyde dehydrogenase 2 family member ISO SMPDB SMP:00139 NCBI chrNW_004936558:4,362,693...4,387,705
Ensembl chrNW_004936558:4,362,654...4,390,414
JBrowse link
G Aldh6a1 aldehyde dehydrogenase 6 family member A1 ISO SMPDB SMP:00139 NCBI chrNW_004936488:3,320,357...3,340,463
Ensembl chrNW_004936488:3,318,422...3,340,626
JBrowse link
G Aox1 aldehyde oxidase 1 ISO SMPDB SMP:00139 NCBI chrNW_004936825:263,725...332,424
Ensembl chrNW_004936825:263,081...332,622
JBrowse link
G Auh AU RNA binding methylglutaconyl-CoA hydratase ISO SMPDB SMP:00139 NCBI chrNW_004936796:160,335...302,458
Ensembl chrNW_004936796:161,003...303,250
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
Ensembl chrNW_004936661:3,799,955...3,819,851
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
Ensembl chrNW_004936510:9,902,670...10,081,587
JBrowse link
G Dbt dihydrolipoamide branched chain transacylase E2 ISO SMPDB SMP:00139 NCBI chrNW_004936748:342,655...386,017
Ensembl chrNW_004936748:342,394...386,000
JBrowse link
G Dld dihydrolipoamide dehydrogenase ISO SMPDB SMP:00139 NCBI chrNW_004936479:16,247,230...16,271,597
Ensembl chrNW_004936479:16,243,829...16,271,616
JBrowse link
G Echs1 enoyl-CoA hydratase, short chain 1 ISO SMPDB SMP:00139 NCBI chrNW_004936486:19,488,619...19,496,583
Ensembl chrNW_004936486:19,488,554...19,496,575
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
Ensembl chrNW_004936506:7,811,600...7,882,642
JBrowse link
G Hmgcl 3-hydroxy-3-methylglutaryl-CoA lyase ISO SMPDB SMP:00139 NCBI chrNW_004936474:8,955,216...8,971,078
Ensembl chrNW_004936474:8,954,379...8,971,132
JBrowse link
G Hmgcs2 3-hydroxy-3-methylglutaryl-CoA synthase 2 ISO SMPDB SMP:00139 NCBI chrNW_004936627:4,451,847...4,473,163
Ensembl chrNW_004936627:4,451,823...4,473,223
JBrowse link
G Hsd17b10 hydroxysteroid 17-beta dehydrogenase 10 ISO SMPDB SMP:00139 NCBI chrNW_004936751:399,743...402,200
Ensembl chrNW_004936751:399,701...402,344
JBrowse link
G Ivd isovaleryl-CoA dehydrogenase ISO SMPDB SMP:00139 NCBI chrNW_004936471:3,871,152...3,883,521
Ensembl chrNW_004936471:3,871,148...3,885,241
JBrowse link
G Mccc1 methylcrotonyl-CoA carboxylase subunit 1 ISO SMPDB SMP:00139 NCBI chrNW_004936566:409,558...468,898
Ensembl chrNW_004936566:409,462...469,225
JBrowse link
G Mccc2 methylcrotonyl-CoA carboxylase subunit 2 ISO SMPDB SMP:00139 NCBI chrNW_004936549:4,760,073...4,830,190
Ensembl chrNW_004936549:4,755,546...4,830,242
JBrowse link
G Mcee methylmalonyl-CoA epimerase ISO SMPDB SMP:00139 NCBI chrNW_004936483:971,148...990,634
Ensembl chrNW_004936483:970,452...990,638
JBrowse link
G Mmut methylmalonyl-CoA mutase ISO SMPDB SMP:00139 NCBI chrNW_004936476:11,208,251...11,241,648
Ensembl chrNW_004936476:11,208,267...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
Ensembl chrNW_004936472:10,164,642...10,501,878
JBrowse link
G Pccb propionyl-CoA carboxylase subunit beta ISO SMPDB SMP:00139 NCBI chrNW_004936529:9,106,525...9,166,311
Ensembl chrNW_004936529:9,106,496...9,176,394
JBrowse link

Term paths to the root
Path 1
Term Annotations click to browse term
  pathway 3951
    disease pathway 688
      congenital disease pathway 507
        inborn genetic disease pathway 507
          inborn error of metabolism pathway 507
            3-methylglutaconic aciduria type 1 pathway 30
paths to the root