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
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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 chr10:6,996,688...7,092,835
Ensembl chr10:6,999,819...7,092,835
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Acaa2
acetyl-CoA acyltransferase 2
ISO
SMPDB
SMP:00200
NCBI chr18:68,345,136...68,373,246
Ensembl chr18:68,345,012...68,373,249
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Acad8
acyl-CoA dehydrogenase family, member 8
ISO
SMPDB
SMP:00200
NCBI chr 8:25,382,271...25,406,404
Ensembl chr 8:25,382,273...25,406,414
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Acadm
acyl-CoA dehydrogenase medium chain
ISO
SMPDB
SMP:00200
NCBI chr 2:242,858,865...242,883,036
Ensembl chr 2:242,858,865...242,883,147
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Acads
acyl-CoA dehydrogenase short chain
ISO
SMPDB
SMP:00200
NCBI chr12:41,493,650...41,502,897
Ensembl chr12:41,493,626...41,502,898
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Acadsb
acyl-CoA dehydrogenase, short/branched chain
ISO
SMPDB
SMP:00200
NCBI chr 1:186,188,939...186,227,796
Ensembl chr 1:186,188,987...186,230,379
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Acat1
acetyl-CoA acetyltransferase 1
ISO
SMPDB
SMP:00200
NCBI chr 8:53,979,813...54,008,861
Ensembl chr 8:53,979,813...54,008,855
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Aldh2
aldehyde dehydrogenase 2 family member
ISO
SMPDB
SMP:00200
NCBI chr12:34,949,549...34,982,527
Ensembl chr12:34,901,219...34,982,521
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Aldh6a1
aldehyde dehydrogenase 6 family, member A1
ISO
SMPDB
SMP:00200
NCBI chr 6:104,077,975...104,098,636
Ensembl chr 6:104,077,979...104,098,656
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Aox1
aldehyde oxidase 1
ISO
SMPDB
SMP:00200
NCBI chr 9:59,579,621...59,658,772
Ensembl chr 9:59,579,649...59,658,770
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Auh
AU RNA binding methylglutaconyl-CoA hydratase
ISO
SMPDB
SMP:00200
NCBI chr17:12,329,522...12,424,882
Ensembl chr17:12,329,524...12,424,896
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Bcat1
branched chain amino acid transaminase 1
ISO
SMPDB
SMP:00200
NCBI chr 4:177,964,834...178,046,573
Ensembl chr 4:177,964,834...178,046,597
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Bckdha
branched chain keto acid dehydrogenase E1 subunit alpha
ISO
SMPDB
SMP:00200
NCBI chr 1:81,138,946...81,167,765
Ensembl chr 1:81,138,947...81,167,862
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Bckdhb
branched chain keto acid dehydrogenase E1 subunit beta
ISO
SMPDB
SMP:00200
NCBI chr 8:84,845,264...85,027,812
Ensembl chr 8:84,845,264...85,027,812
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Dbt
dihydrolipoamide branched chain transacylase E2
ISO
SMPDB
SMP:00200
NCBI chr 2:204,481,744...204,510,612
Ensembl chr 2:204,481,737...204,510,609
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Dld
dihydrolipoamide dehydrogenase
ISO
SMPDB
SMP:00200
NCBI chr 6:47,904,153...47,924,814
Ensembl chr 6:47,903,914...47,924,795
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Echs1
enoyl-CoA hydratase, short chain 1
ISO
SMPDB
SMP:00200
NCBI chr 1:194,895,036...194,903,863
Ensembl chr 1:194,895,036...194,903,884
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Hibadh
3-hydroxyisobutyrate dehydrogenase
ISO
SMPDB
SMP:00200
NCBI chr 4:81,649,180...81,747,244
Ensembl chr 4:81,649,175...81,780,617
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Hibch
3-hydroxyisobutyryl-CoA hydrolase
ISO
SMPDB
SMP:00200
NCBI chr 9:48,590,097...48,669,896
Ensembl chr 9:48,590,099...48,669,824
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Hmgcl
3-hydroxy-3-methylglutaryl-CoA lyase
ISO
SMPDB
SMP:00200
NCBI chr 5:148,178,203...148,192,072
Ensembl chr 5:148,178,252...148,192,068
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Hmgcs2
3-hydroxy-3-methylglutaryl-CoA synthase 2
ISO
SMPDB
SMP:00200
NCBI chr 2:185,875,609...185,903,505
Ensembl chr 2:185,875,616...185,902,130
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Hsd17b10
hydroxysteroid (17-beta) dehydrogenase 10
ISO
SMPDB
SMP:00200
NCBI chr X:21,089,142...21,091,603
Ensembl chr X:21,089,122...21,109,488
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Ivd
isovaleryl-CoA dehydrogenase
ISO
SMPDB
SMP:00200
NCBI chr 3:105,851,710...105,872,144
Ensembl chr 3:105,851,683...105,872,575
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Mccc1
methylcrotonyl-CoA carboxylase subunit 1
ISO
SMPDB
SMP:00200
NCBI chr 2:118,799,147...118,851,181
Ensembl chr 2:118,799,150...118,851,222
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Mccc2
methylcrotonyl-CoA carboxylase subunit 2
ISO
SMPDB
SMP:00200
NCBI chr 2:31,304,927...31,375,978
Ensembl chr 2:31,304,932...31,375,972
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Mcee
methylmalonyl CoA epimerase
ISO
SMPDB
SMP:00200
NCBI chr 1:117,964,576...117,987,779
Ensembl chr 1:117,964,576...117,988,484
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Mmut
methylmalonyl-CoA mutase
ISO
SMPDB
SMP:00200
NCBI chr 9:19,928,720...19,956,985
Ensembl chr 9:19,928,727...19,957,046
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Oxct1
3-oxoacid CoA transferase 1
ISO
SMPDB
SMP:00200
NCBI chr 2:53,236,370...53,384,715
Ensembl chr 2:53,236,368...53,384,714
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Pcca
propionyl-CoA carboxylase subunit alpha
ISO
SMPDB
SMP:00200
NCBI chr15:99,627,955...99,969,555
Ensembl chr15:99,627,982...99,968,266
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Pccb
propionyl-CoA carboxylase subunit beta
ISO
SMPDB
SMP:00200
NCBI chr 8:101,591,218...101,641,213
Ensembl chr 8:101,590,737...101,641,234
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Abat
4-aminobutyrate aminotransferase
ISO
SMPDB
SMP:00201
NCBI chr10:6,996,688...7,092,835
Ensembl chr10:6,999,819...7,092,835
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Acaca
acetyl-CoA carboxylase alpha
ISO
SMPDB
SMP:00201
NCBI chr10:69,014,261...69,276,453
Ensembl chr10:69,014,170...69,276,457
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Acadm
acyl-CoA dehydrogenase medium chain
ISO
SMPDB
SMP:00201
NCBI chr 2:242,858,865...242,883,036
Ensembl chr 2:242,858,865...242,883,147
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Acat1
acetyl-CoA acetyltransferase 1
ISO
SMPDB
SMP:00201
NCBI chr 8:53,979,813...54,008,861
Ensembl chr 8:53,979,813...54,008,855
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Acss1
acyl-CoA synthetase short-chain family member 1
ISO
SMPDB
SMP:00201
NCBI chr 3:139,450,383...139,500,325
Ensembl chr 3:139,450,383...139,500,325
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Acss3
acyl-CoA synthetase short-chain family member 3
ISO
SMPDB
SMP:00201
NCBI chr 7:42,241,261...42,450,209
Ensembl chr 7:42,242,652...42,450,230
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Aldh6a1
aldehyde dehydrogenase 6 family, member A1
ISO
SMPDB
SMP:00201
NCBI chr 6:104,077,975...104,098,636
Ensembl chr 6:104,077,979...104,098,656
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Bckdha
branched chain keto acid dehydrogenase E1 subunit alpha
ISO
SMPDB
SMP:00201
NCBI chr 1:81,138,946...81,167,765
Ensembl chr 1:81,138,947...81,167,862
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Bckdhb
branched chain keto acid dehydrogenase E1 subunit beta
ISO
SMPDB
SMP:00201
NCBI chr 8:84,845,264...85,027,812
Ensembl chr 8:84,845,264...85,027,812
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Dbt
dihydrolipoamide branched chain transacylase E2
ISO
SMPDB
SMP:00201
NCBI chr 2:204,481,744...204,510,612
Ensembl chr 2:204,481,737...204,510,609
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Dld
dihydrolipoamide dehydrogenase
ISO
SMPDB
SMP:00201
NCBI chr 6:47,904,153...47,924,814
Ensembl chr 6:47,903,914...47,924,795
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Echs1
enoyl-CoA hydratase, short chain 1
ISO
SMPDB
SMP:00201
NCBI chr 1:194,895,036...194,903,863
Ensembl chr 1:194,895,036...194,903,884
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Hibch
3-hydroxyisobutyryl-CoA hydrolase
ISO
SMPDB
SMP:00201
NCBI chr 9:48,590,097...48,669,896
Ensembl chr 9:48,590,099...48,669,824
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Ldhal6b
lactate dehydrogenase A-like 6B
ISO
SMPDB
SMP:00201
NCBI chr 1:45,997,845...45,999,268
Ensembl chr 1:45,991,991...45,999,272
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Mcee
methylmalonyl CoA epimerase
ISO
SMPDB
SMP:00201
NCBI chr 1:117,964,576...117,987,779
Ensembl chr 1:117,964,576...117,988,484
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Mlycd
malonyl-CoA decarboxylase
ISO
SMPDB
SMP:00201
NCBI chr19:47,447,931...47,463,794
Ensembl chr19:47,447,970...47,463,793
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Mmut
methylmalonyl-CoA mutase
ISO
SMPDB
SMP:00201
NCBI chr 9:19,928,720...19,956,985
Ensembl chr 9:19,928,727...19,957,046
G
Pcca
propionyl-CoA carboxylase subunit alpha
ISO
SMPDB
SMP:00201
NCBI chr15:99,627,955...99,969,555
Ensembl chr15:99,627,982...99,968,266
G
Pccb
propionyl-CoA carboxylase subunit beta
ISO
SMPDB
SMP:00201
NCBI chr 8:101,591,218...101,641,213
Ensembl chr 8:101,590,737...101,641,234
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