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
Accession: PW:0002274
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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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
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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
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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
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Acadm
acyl-CoA dehydrogenase medium chain
ISO
SMPDB
SMP:00139
NCBI chrNW_004936571:2,761,254...2,797,045
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Acads
acyl-CoA dehydrogenase short chain
ISO
SMPDB
SMP:00139
NCBI chrNW_004936668:257,214...272,089
Ensembl chrNW_004936668:254,595...272,118
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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
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Acat1
acetyl-CoA acetyltransferase 1
ISO
SMPDB
SMP:00139
NCBI chrNW_004936551:594,647...626,016
Ensembl chrNW_004936551:591,695...626,056
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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
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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
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Aox1
aldehyde oxidase 1
ISO
SMPDB
SMP:00139
NCBI chrNW_004936825:263,725...332,424
Ensembl chrNW_004936825:263,081...332,622
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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
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Bcat1
branched chain amino acid transaminase 1
ISO
SMPDB
SMP:00139
NCBI chrNW_004936548:2,385,756...2,454,423
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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
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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
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Dbt
dihydrolipoamide branched chain transacylase E2
ISO
SMPDB
SMP:00139
NCBI chrNW_004936748:342,655...386,017
Ensembl chrNW_004936748:342,394...386,000
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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
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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
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Hibadh
3-hydroxyisobutyrate dehydrogenase
ISO
SMPDB
SMP:00139
NCBI chrNW_004936478:4,118,752...4,219,908
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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
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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
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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
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Hsd17b10
hydroxysteroid 17-beta dehydrogenase 10
ISO
SMPDB
SMP:00139
NCBI chrNW_004936751:399,743...402,200
Ensembl chrNW_004936751:399,701...402,344
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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
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Mccc1
methylcrotonyl-CoA carboxylase subunit 1
ISO
SMPDB
SMP:00139
NCBI chrNW_004936566:409,558...468,898
Ensembl chrNW_004936566:409,462...469,225
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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
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Mcee
methylmalonyl-CoA epimerase
ISO
SMPDB
SMP:00139
NCBI chrNW_004936483:971,148...990,634
Ensembl chrNW_004936483:970,452...990,638
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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
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Oxct1
3-oxoacid CoA-transferase 1
ISO
SMPDB
SMP:00139
NCBI chrNW_004936518:924,840...1,057,201
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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
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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
Term paths to the root one shortest all shortest one longest all longest one shortest and longest all