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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:malonic aciduria pathway
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Accession:PW:0002289 term browser browse the term
Definition:A rare inherited metabolic condition due to alterations in the activity of malonyl-CoA decarboxylase.
Synonyms:exact_synonym: malonic aciduria disease pathway
 related_synonym: SMP:00198;   SMP:00502;   malonyl-CoA decarboxylase deficiency pathway
 alt_id: PW:0002550

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malonic aciduria pathway term browser
Symbol Object Name Evidence Notes Source PubMed Reference(s) RGD Reference(s) Position
G Abat 4-aminobutyrate aminotransferase ISO SMPDB SMP:00198 SMP:00502 NCBI chr10:7,093,406...7,200,439
Ensembl chr10:7,093,405...7,200,499
JBrowse link
G Acaca acetyl-CoA carboxylase alpha ISO SMPDB SMP:00198 SMP:00502 NCBI chr10:71,519,392...71,719,910
Ensembl chr10:71,536,533...71,719,910
JBrowse link
G Acadm acyl-CoA dehydrogenase medium chain ISO SMPDB SMP:00198 SMP:00502 NCBI chr 2:260,124,418...260,148,589
Ensembl chr 2:260,124,418...260,148,589
JBrowse link
G Acat1 acetyl-CoA acetyltransferase 1 ISO SMPDB SMP:00198 SMP:00502 NCBI chr 8:58,166,990...58,195,884
Ensembl chr 8:58,166,990...58,195,884
JBrowse link
G Acss1 acyl-CoA synthetase short-chain family member 1 ISO SMPDB SMP:00198 SMP:00502 NCBI chr 3:146,420,346...146,470,293
Ensembl chr 3:146,420,346...146,470,293
JBrowse link
G Acss3 acyl-CoA synthetase short-chain family member 3 ISO SMPDB SMP:00198 SMP:00502 NCBI chr 7:49,047,022...49,250,953
Ensembl chr 7:49,047,013...49,250,957
JBrowse link
G Aldh6a1 aldehyde dehydrogenase 6 family, member A1 ISO SMPDB SMP:00198 SMP:00502 NCBI chr 6:108,146,552...108,167,185
Ensembl chr 6:108,146,582...108,167,185
JBrowse link
G Bckdha branched chain keto acid dehydrogenase E1 subunit alpha ISO SMPDB SMP:00198 SMP:00502 NCBI chr 1:82,423,291...82,452,094
Ensembl chr 1:82,423,277...82,452,281
JBrowse link
G Bckdhb branched chain keto acid dehydrogenase E1 subunit beta ISO SMPDB SMP:00198 SMP:00502 NCBI chr 8:91,464,229...91,656,134
Ensembl chr 8:91,464,229...91,656,134
JBrowse link
G Dbt dihydrolipoamide branched chain transacylase E2 ISO SMPDB SMP:00198 SMP:00502 NCBI chr 2:219,563,783...219,592,651
Ensembl chr 2:219,563,783...219,592,650
JBrowse link
G Dld dihydrolipoamide dehydrogenase ISO SMPDB SMP:00198 SMP:00502 NCBI chr 6:50,597,677...50,618,694
Ensembl chr 6:50,597,677...50,618,694
JBrowse link
G Echs1 enoyl-CoA hydratase, short chain 1 ISO SMPDB SMP:00198 SMP:00502 NCBI chr 1:212,570,213...212,579,040
Ensembl chr 1:212,570,195...212,579,057
Ensembl chr 1:212,570,195...212,579,057
JBrowse link
G Hibch 3-hydroxyisobutyryl-CoA hydrolase ISO SMPDB SMP:00198 SMP:00502 NCBI chr 9:53,446,185...53,526,727
Ensembl chr 9:53,446,194...53,526,728
JBrowse link
G Ldhal6b lactate dehydrogenase A-like 6B ISO SMPDB SMP:00198 SMP:00502 NCBI chr 1:46,307,853...46,309,276 JBrowse link
G Mcee methylmalonyl CoA epimerase ISO SMPDB SMP:00198 SMP:00502 NCBI chr 1:125,229,487...125,252,692
Ensembl chr 1:125,229,469...125,252,666
JBrowse link
G Mlycd malonyl-CoA decarboxylase ISO SMPDB SMP:00198 SMP:00502 NCBI chr19:52,032,950...52,048,773
Ensembl chr19:52,032,886...52,048,753
JBrowse link
G Mmut methylmalonyl-CoA mutase ISO SMPDB SMP:00198 SMP:00502 NCBI chr 9:23,323,934...23,352,144
Ensembl chr 9:23,323,936...23,352,668
JBrowse link
G Pcca propionyl-CoA carboxylase subunit alpha ISO SMPDB SMP:00198 SMP:00502 NCBI chr15:108,960,509...109,306,879
Ensembl chr15:108,960,562...109,306,603
JBrowse link
G Pccb propionyl-CoA carboxylase subunit beta ISO SMPDB SMP:00198 SMP:00502 NCBI chr 8:109,368,887...109,418,871
Ensembl chr 8:109,368,624...109,418,872
JBrowse link

Term paths to the root
Path 1
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  pathway 6103
    disease pathway 1957
      congenital disease pathway 529
        inborn genetic disease pathway 529
          inborn error of metabolism pathway 529
            malonic aciduria pathway 19
paths to the root