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: methylmalonic acidemia pathway
Accession: PW:0001810
browse the term
Definition: An inherited condition resulting from alterations in the metabolic pathway whereby methylmalonyl-coenzyme A (CoA) is converted into succinyl-CoA.
Synonyms: exact_synonym: methylmalonic acidemia disease pathway
related_synonym: SMP:00200; methylmalonic aciduria disease pathway
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Abat
4-aminobutyrate aminotransferase
ISO
SMPDB
SMP:00200
NCBI chr10:7,093,406...7,200,439
Ensembl chr10:7,093,405...7,200,499
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Acaa2
acetyl-CoA acyltransferase 2
ISO
SMPDB
SMP:00200
NCBI chr18:70,733,872...70,762,395
Ensembl chr18:70,733,872...70,762,395
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Acad8
acyl-CoA dehydrogenase family, member 8
ISO
SMPDB
SMP:00200
NCBI chr 8:28,023,401...28,044,967
Ensembl chr 8:28,024,123...28,044,876
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Acadm
acyl-CoA dehydrogenase medium chain
ISO
SMPDB
SMP:00200
NCBI chr 2:260,124,418...260,148,589
Ensembl chr 2:260,124,418...260,148,589
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Acads
acyl-CoA dehydrogenase short chain
ISO
SMPDB
SMP:00200
NCBI chr12:47,254,503...47,263,747
Ensembl chr12:47,254,484...47,263,747
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Acadsb
acyl-CoA dehydrogenase, short/branched chain
ISO
SMPDB
SMP:00200
NCBI chr 1:201,981,362...202,022,771
Ensembl chr 1:201,981,357...202,021,008
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Acat1
acetyl-CoA acetyltransferase 1
ISO
SMPDB
SMP:00200
NCBI chr 8:58,166,990...58,195,884
Ensembl chr 8:58,166,990...58,195,884
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Aldh2
aldehyde dehydrogenase 2 family member
ISO
SMPDB
SMP:00200
NCBI chr12:40,466,418...40,498,813
Ensembl chr12:40,466,495...40,498,752
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Aldh6a1
aldehyde dehydrogenase 6 family, member A1
ISO
SMPDB
SMP:00200
NCBI chr 6:108,146,552...108,167,185
Ensembl chr 6:108,146,582...108,167,185
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Aox1
aldehyde oxidase 1
ISO
SMPDB
SMP:00200
NCBI chr 9:64,929,682...65,007,872
Ensembl chr 9:64,929,721...65,007,870
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Auh
AU RNA binding methylglutaconyl-CoA hydratase
ISO
SMPDB
SMP:00200
NCBI chr17:12,310,178...12,405,224
Ensembl chr17:12,310,214...12,405,205
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Bcat1
branched chain amino acid transaminase 1
ISO
SMPDB
SMP:00200
NCBI chr 4:179,259,305...179,340,021
Ensembl chr 4:179,259,308...179,339,795
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Bckdha
branched chain keto acid dehydrogenase E1 subunit alpha
ISO
SMPDB
SMP:00200
NCBI chr 1:82,423,291...82,452,094
Ensembl chr 1:82,423,277...82,452,281
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Bckdhb
branched chain keto acid dehydrogenase E1 subunit beta
ISO
SMPDB
SMP:00200
NCBI chr 8:91,464,229...91,656,134
Ensembl chr 8:91,464,229...91,656,134
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Dbt
dihydrolipoamide branched chain transacylase E2
ISO
SMPDB
SMP:00200
NCBI chr 2:219,563,783...219,592,651
Ensembl chr 2:219,563,783...219,592,650
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Dld
dihydrolipoamide dehydrogenase
ISO
SMPDB
SMP:00200
NCBI chr 6:50,597,677...50,618,694
Ensembl chr 6:50,597,677...50,618,694
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Echs1
enoyl-CoA hydratase, short chain 1
ISO
SMPDB
SMP:00200
NCBI chr 1:212,570,213...212,579,040
Ensembl chr 1:212,570,195...212,579,057
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Hibadh
3-hydroxyisobutyrate dehydrogenase
ISO
SMPDB
SMP:00200
NCBI chr 4:82,604,369...82,702,429
Ensembl chr 4:82,604,373...82,702,429
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Hibch
3-hydroxyisobutyryl-CoA hydrolase
ISO
SMPDB
SMP:00200
NCBI chr 9:53,446,185...53,526,727
Ensembl chr 9:53,446,194...53,526,728
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Hmgcl
3-hydroxy-3-methylglutaryl-CoA lyase
ISO
SMPDB
SMP:00200
NCBI chr 5:154,294,841...154,308,582
Ensembl chr 5:154,294,806...154,308,640
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Hmgcs2
3-hydroxy-3-methylglutaryl-CoA synthase 2
ISO
SMPDB
SMP:00200
NCBI chr 2:200,452,623...200,480,785
Ensembl chr 2:200,452,624...200,479,423
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Hsd17b10
hydroxysteroid (17-beta) dehydrogenase 10
ISO
SMPDB
SMP:00200
NCBI chr X:21,696,796...21,699,241
Ensembl chr X:21,696,772...21,699,241
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Ivd
isovaleryl-CoA dehydrogenase
ISO
SMPDB
SMP:00200
NCBI chr 3:110,669,355...110,689,789
Ensembl chr 3:110,669,312...110,690,173
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Mccc1
methylcrotonoyl-CoA carboxylase 1
ISO
SMPDB
SMP:00200
NCBI chr 2:122,550,777...122,690,540
Ensembl chr 2:122,550,775...122,690,617
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Mccc2
methylcrotonoyl-CoA carboxylase 2
ISO
SMPDB
SMP:00200
NCBI chr 2:30,175,017...30,246,028
Ensembl chr 2:30,175,018...30,246,010
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Mcee
methylmalonyl CoA epimerase
ISO
SMPDB
SMP:00200
NCBI chr 1:125,229,487...125,252,692
Ensembl chr 1:125,229,469...125,252,666
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Mmut
methylmalonyl-CoA mutase
ISO
SMPDB
SMP:00200
NCBI chr 9:23,323,934...23,352,144
Ensembl chr 9:23,323,936...23,352,668
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Oxct1
3-oxoacid CoA transferase 1
ISO
SMPDB
SMP:00200
NCBI chr 2:53,859,738...54,007,733
Ensembl chr 2:53,859,737...54,007,756
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Pcca
propionyl-CoA carboxylase subunit alpha
ISO
SMPDB
SMP:00200
NCBI chr15:108,960,509...109,306,879
Ensembl chr15:108,960,562...109,306,603
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Pccb
propionyl-CoA carboxylase subunit beta
ISO
SMPDB
SMP:00200
NCBI chr 8:109,368,887...109,418,871
Ensembl chr 8:109,368,624...109,418,872
G
Abat
4-aminobutyrate aminotransferase
ISO
SMPDB
SMP:00201
NCBI chr10:7,093,406...7,200,439
Ensembl chr10:7,093,405...7,200,499
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Acaca
acetyl-CoA carboxylase alpha
ISO
SMPDB
SMP:00201
NCBI chr10:71,519,392...71,719,910
Ensembl chr10:71,536,533...71,719,910
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Acadm
acyl-CoA dehydrogenase medium chain
ISO
SMPDB
SMP:00201
NCBI chr 2:260,124,418...260,148,589
Ensembl chr 2:260,124,418...260,148,589
G
Acat1
acetyl-CoA acetyltransferase 1
ISO
SMPDB
SMP:00201
NCBI chr 8:58,166,990...58,195,884
Ensembl chr 8:58,166,990...58,195,884
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Acss1
acyl-CoA synthetase short-chain family member 1
ISO
SMPDB
SMP:00201
NCBI chr 3:146,420,346...146,470,293
Ensembl chr 3:146,420,346...146,470,293
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Acss3
acyl-CoA synthetase short-chain family member 3
ISO
SMPDB
SMP:00201
NCBI chr 7:49,047,022...49,250,953
Ensembl chr 7:49,047,013...49,250,957
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Aldh6a1
aldehyde dehydrogenase 6 family, member A1
ISO
SMPDB
SMP:00201
NCBI chr 6:108,146,552...108,167,185
Ensembl chr 6:108,146,582...108,167,185
G
Bckdha
branched chain keto acid dehydrogenase E1 subunit alpha
ISO
SMPDB
SMP:00201
NCBI chr 1:82,423,291...82,452,094
Ensembl chr 1:82,423,277...82,452,281
G
Bckdhb
branched chain keto acid dehydrogenase E1 subunit beta
ISO
SMPDB
SMP:00201
NCBI chr 8:91,464,229...91,656,134
Ensembl chr 8:91,464,229...91,656,134
G
Dbt
dihydrolipoamide branched chain transacylase E2
ISO
SMPDB
SMP:00201
NCBI chr 2:219,563,783...219,592,651
Ensembl chr 2:219,563,783...219,592,650
G
Dld
dihydrolipoamide dehydrogenase
ISO
SMPDB
SMP:00201
NCBI chr 6:50,597,677...50,618,694
Ensembl chr 6:50,597,677...50,618,694
G
Echs1
enoyl-CoA hydratase, short chain 1
ISO
SMPDB
SMP:00201
NCBI chr 1:212,570,213...212,579,040
Ensembl chr 1:212,570,195...212,579,057
G
Hibch
3-hydroxyisobutyryl-CoA hydrolase
ISO
SMPDB
SMP:00201
NCBI chr 9:53,446,185...53,526,727
Ensembl chr 9:53,446,194...53,526,728
G
Ldhal6b
lactate dehydrogenase A-like 6B
ISO
SMPDB
SMP:00201
NCBI chr 1:46,307,853...46,309,276
G
Mcee
methylmalonyl CoA epimerase
ISO
SMPDB
SMP:00201
NCBI chr 1:125,229,487...125,252,692
Ensembl chr 1:125,229,469...125,252,666
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Mlycd
malonyl-CoA decarboxylase
ISO
SMPDB
SMP:00201
NCBI chr19:52,032,950...52,048,773
Ensembl chr19:52,032,886...52,048,753
G
Mmut
methylmalonyl-CoA mutase
ISO
SMPDB
SMP:00201
NCBI chr 9:23,323,934...23,352,144
Ensembl chr 9:23,323,936...23,352,668
G
Pcca
propionyl-CoA carboxylase subunit alpha
ISO
SMPDB
SMP:00201
NCBI chr15:108,960,509...109,306,879
Ensembl chr15:108,960,562...109,306,603
G
Pccb
propionyl-CoA carboxylase subunit beta
ISO
SMPDB
SMP:00201
NCBI chr 8:109,368,887...109,418,871
Ensembl chr 8:109,368,624...109,418,872
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