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 (https://rgd.mcw.edu/rgdweb/contact/contactus.html ).
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
Symbol
Object Name
Evidence
Notes
Source
PubMed Reference(s)
RGD Reference(s)
Position
G
Abat
4-aminobutyrate aminotransferase
ISO
SMPDB
SMP:00200
NCBI chr10:7,503,351...7,599,474
Ensembl chr10:6,999,819...7,092,835
G
Acaa2
acetyl-CoA acyltransferase 2
ISO
SMPDB
SMP:00200
NCBI chr18:70,620,310...70,648,417
Ensembl chr18:68,345,012...68,373,249
G
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
G
Acadm
acyl-CoA dehydrogenase medium chain
ISO
SMPDB
SMP:00200
NCBI chr 2:245,518,693...245,542,864
Ensembl chr 2:242,858,865...242,883,147
G
Acads
acyl-CoA dehydrogenase short chain
ISO
SMPDB
SMP:00200
NCBI chr12:47,154,259...47,163,580
Ensembl chr12:41,493,626...41,502,898
G
Acadsb
acyl-CoA dehydrogenase, short/branched chain
ISO
SMPDB
SMP:00200
NCBI chr 1:195,619,088...195,660,564
Ensembl chr 1:186,188,987...186,230,379
G
Acat1
acetyl-CoA acetyltransferase 1
ISO
SMPDB
SMP:00200
NCBI chr 8:62,876,003...62,905,080
Ensembl chr 8:53,979,813...54,008,855
G
Aldh2
aldehyde dehydrogenase 2 family member
ISO
SMPDB
SMP:00200
NCBI chr12:40,610,244...40,643,220
Ensembl chr12:34,901,219...34,982,521
G
Aldh6a1
aldehyde dehydrogenase 6 family, member A1
ISO
SMPDB
SMP:00200
NCBI chr 6:109,809,092...109,829,725
Ensembl chr 6:104,077,979...104,098,656
G
Aox1
aldehyde oxidase 1
ISO
SMPDB
SMP:00200
NCBI chr 9:67,073,831...67,152,980
Ensembl chr 9:59,579,649...59,658,770
G
Auh
AU RNA binding methylglutaconyl-CoA hydratase
ISO
SMPDB
SMP:00200
NCBI chr17:12,481,396...12,576,748
Ensembl chr17:12,329,524...12,424,896
G
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
G
Bckdha
branched chain keto acid dehydrogenase E1 subunit alpha
ISO
SMPDB
SMP:00200
NCBI chr 1:90,266,731...90,295,521
Ensembl chr 1:81,138,947...81,167,862
G
Bckdhb
branched chain keto acid dehydrogenase E1 subunit beta
ISO
SMPDB
SMP:00200
NCBI chr 8:93,725,277...93,907,799
Ensembl chr 8:84,845,264...85,027,812
G
Dbt
dihydrolipoamide branched chain transacylase E2
ISO
SMPDB
SMP:00200
NCBI chr 2:207,166,660...207,195,528
Ensembl chr 2:204,481,737...204,510,609
G
Dld
dihydrolipoamide dehydrogenase
ISO
SMPDB
SMP:00200
NCBI chr 6:53,631,686...53,655,059
Ensembl chr 6:47,903,914...47,924,795
G
Echs1
enoyl-CoA hydratase, short chain 1
ISO
SMPDB
SMP:00200
NCBI chr 1:204,324,679...204,333,506
Ensembl chr 1:194,895,036...194,903,884
G
Hibadh
3-hydroxyisobutyrate dehydrogenase
ISO
SMPDB
SMP:00200
NCBI chr 4:82,979,759...83,077,819
Ensembl chr 4:81,649,175...81,780,617
G
Hibch
3-hydroxyisobutyryl-CoA hydrolase
ISO
SMPDB
SMP:00200
NCBI chr 9:56,082,082...56,161,796
Ensembl chr 9:48,590,099...48,669,824
G
Hmgcl
3-hydroxy-3-methylglutaryl-CoA lyase
ISO
SMPDB
SMP:00200
NCBI chr 5:153,461,738...153,475,552
Ensembl chr 5:148,178,252...148,192,068
G
Hmgcs2
3-hydroxy-3-methylglutaryl-CoA synthase 2
ISO
SMPDB
SMP:00200
NCBI chr 2:188,564,348...188,590,872
Ensembl chr 2:185,875,616...185,902,130
G
Hsd17b10
hydroxysteroid (17-beta) dehydrogenase 10
ISO
SMPDB
SMP:00200
NCBI chr X:24,568,551...24,571,012
Ensembl chr X:21,089,122...21,109,488
G
Ivd
isovaleryl-CoA dehydrogenase
ISO
SMPDB
SMP:00200
NCBI chr 3:126,305,584...126,326,016
Ensembl chr 3:105,851,683...105,872,575
G
Mccc1
methylcrotonyl-CoA carboxylase subunit 1
ISO
SMPDB
SMP:00200
NCBI chr 2:120,727,313...120,779,334
Ensembl chr 2:118,799,150...118,851,222
G
Mccc2
methylcrotonyl-CoA carboxylase subunit 2
ISO
SMPDB
SMP:00200
NCBI chr 2:33,039,044...33,110,092
Ensembl chr 2:31,304,932...31,375,972
G
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
G
Mmut
methylmalonyl-CoA mutase
ISO
SMPDB
SMP:00200
NCBI chr 9:27,425,935...27,454,202
Ensembl chr 9:19,928,727...19,957,046
G
Oxct1
3-oxoacid CoA transferase 1
ISO
SMPDB
SMP:00200
NCBI chr 2:54,963,964...55,112,303
Ensembl chr 2:53,236,368...53,384,714
G
Pcca
propionyl-CoA carboxylase subunit alpha
ISO
SMPDB
SMP:00200
NCBI chr15:106,034,586...106,374,908
Ensembl chr15:99,627,982...99,968,266
G
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
Symbol
Object Name
Evidence
Notes
Source
PubMed Reference(s)
RGD Reference(s)
Position
G
Abat
4-aminobutyrate aminotransferase
ISO
SMPDB
SMP:00201
NCBI chr10:7,503,351...7,599,474
Ensembl chr10:6,999,819...7,092,835
G
Acaca
acetyl-CoA carboxylase alpha
ISO
SMPDB
SMP:00201
NCBI chr10:69,511,627...69,773,888
Ensembl chr10:69,014,170...69,276,457
G
Acadm
acyl-CoA dehydrogenase medium chain
ISO
SMPDB
SMP:00201
NCBI chr 2:245,518,693...245,542,864
Ensembl chr 2:242,858,865...242,883,147
G
Acat1
acetyl-CoA acetyltransferase 1
ISO
SMPDB
SMP:00201
NCBI chr 8:62,876,003...62,905,080
Ensembl chr 8:53,979,813...54,008,855
G
Acss1
acyl-CoA synthetase short-chain family member 1
ISO
SMPDB
SMP:00201
NCBI chr 3:159,910,809...159,960,748
Ensembl chr 3:139,450,383...139,500,325
G
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
G
Aldh6a1
aldehyde dehydrogenase 6 family, member A1
ISO
SMPDB
SMP:00201
NCBI chr 6:109,809,092...109,829,725
Ensembl chr 6:104,077,979...104,098,656
G
Bckdha
branched chain keto acid dehydrogenase E1 subunit alpha
ISO
SMPDB
SMP:00201
NCBI chr 1:90,266,731...90,295,521
Ensembl chr 1:81,138,947...81,167,862
G
Bckdhb
branched chain keto acid dehydrogenase E1 subunit beta
ISO
SMPDB
SMP:00201
NCBI chr 8:93,725,277...93,907,799
Ensembl chr 8:84,845,264...85,027,812
G
Dbt
dihydrolipoamide branched chain transacylase E2
ISO
SMPDB
SMP:00201
NCBI chr 2:207,166,660...207,195,528
Ensembl chr 2:204,481,737...204,510,609
G
Dld
dihydrolipoamide dehydrogenase
ISO
SMPDB
SMP:00201
NCBI chr 6:53,631,686...53,655,059
Ensembl chr 6:47,903,914...47,924,795
G
Echs1
enoyl-CoA hydratase, short chain 1
ISO
SMPDB
SMP:00201
NCBI chr 1:204,324,679...204,333,506
Ensembl chr 1:194,895,036...194,903,884
G
Hibch
3-hydroxyisobutyryl-CoA hydrolase
ISO
SMPDB
SMP:00201
NCBI chr 9:56,082,082...56,161,796
Ensembl chr 9:48,590,099...48,669,824
G
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
G
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
G
Mlycd
malonyl-CoA decarboxylase
ISO
SMPDB
SMP:00201
NCBI chr19:64,356,582...64,372,447
Ensembl chr19:47,447,970...47,463,793
G
Mmut
methylmalonyl-CoA mutase
ISO
SMPDB
SMP:00201
NCBI chr 9:27,425,935...27,454,202
Ensembl chr 9:19,928,727...19,957,046
G
Pcca
propionyl-CoA carboxylase subunit alpha
ISO
SMPDB
SMP:00201
NCBI chr15:106,034,586...106,374,908
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
Term paths to the root one shortest all shortest one longest all longest one shortest and longest all