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-hydroxy-3-methylglutaryl-CoA lyase deficiency pathway
Accession: PW:0002323
browse the term
Definition: A rare autosomal condition resulting from alterations in several metabolic pathways and due to defects in the HMGCL gene.
Synonyms: related_synonym: SMP:00138
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Abat
4-aminobutyrate aminotransferase
ISO
SMPDB
SMP:00138
NCBI chr10:7,503,351...7,599,474
Ensembl chr10:6,999,819...7,092,835
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Acaa2
acetyl-CoA acyltransferase 2
ISO
SMPDB
SMP:00138
NCBI chr18:70,620,310...70,648,417
Ensembl chr18:68,345,012...68,373,249
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Acad8
acyl-CoA dehydrogenase family, member 8
ISO
SMPDB
SMP:00138
NCBI chr 8:33,641,420...33,665,542
Ensembl chr 8:25,382,273...25,406,414
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Acadm
acyl-CoA dehydrogenase medium chain
ISO
SMPDB
SMP:00138
NCBI chr 2:245,518,693...245,542,864
Ensembl chr 2:242,858,865...242,883,147
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Acads
acyl-CoA dehydrogenase short chain
ISO
SMPDB
SMP:00138
NCBI chr12:47,154,259...47,163,580
Ensembl chr12:41,493,626...41,502,898
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Acadsb
acyl-CoA dehydrogenase, short/branched chain
ISO
SMPDB
SMP:00138
NCBI chr 1:195,619,088...195,660,564
Ensembl chr 1:186,188,987...186,230,379
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Acat1
acetyl-CoA acetyltransferase 1
ISO
SMPDB
SMP:00138
NCBI chr 8:62,876,003...62,905,080
Ensembl chr 8:53,979,813...54,008,855
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Aldh2
aldehyde dehydrogenase 2 family member
ISO
SMPDB
SMP:00138
NCBI chr12:40,610,244...40,643,220
Ensembl chr12:34,901,219...34,982,521
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Aldh6a1
aldehyde dehydrogenase 6 family, member A1
ISO
SMPDB
SMP:00138
NCBI chr 6:109,809,092...109,829,725
Ensembl chr 6:104,077,979...104,098,656
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Aox1
aldehyde oxidase 1
ISO
SMPDB
SMP:00138
NCBI chr 9:67,073,831...67,152,980
Ensembl chr 9:59,579,649...59,658,770
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Auh
AU RNA binding methylglutaconyl-CoA hydratase
ISO
SMPDB
SMP:00138
NCBI chr17:12,481,396...12,576,748
Ensembl chr17:12,329,524...12,424,896
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Bcat1
branched chain amino acid transaminase 1
ISO
SMPDB
SMP:00138
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:00138
NCBI chr 1:90,266,731...90,295,521
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:00138
NCBI chr 8:93,725,277...93,907,799
Ensembl chr 8:84,845,264...85,027,812
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Dbt
dihydrolipoamide branched chain transacylase E2
ISO
SMPDB
SMP:00138
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:00138
NCBI chr 6:53,631,686...53,655,059
Ensembl chr 6:47,903,914...47,924,795
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Echs1
enoyl-CoA hydratase, short chain 1
ISO
SMPDB
SMP:00138
NCBI chr 1:204,324,679...204,333,506
Ensembl chr 1:194,895,036...194,903,884
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Hibadh
3-hydroxyisobutyrate dehydrogenase
ISO
SMPDB
SMP:00138
NCBI chr 4:82,979,759...83,077,819
Ensembl chr 4:81,649,175...81,780,617
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Hibch
3-hydroxyisobutyryl-CoA hydrolase
ISO
SMPDB
SMP:00138
NCBI chr 9:56,082,082...56,161,796
Ensembl chr 9:48,590,099...48,669,824
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Hmgcl
3-hydroxy-3-methylglutaryl-CoA lyase
ISO
SMPDB
SMP:00138
NCBI chr 5:153,461,738...153,475,552
Ensembl chr 5:148,178,252...148,192,068
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Hmgcs2
3-hydroxy-3-methylglutaryl-CoA synthase 2
ISO
SMPDB
SMP:00138
NCBI chr 2:188,564,348...188,590,872
Ensembl chr 2:185,875,616...185,902,130
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Hsd17b10
hydroxysteroid (17-beta) dehydrogenase 10
ISO
SMPDB
SMP:00138
NCBI chr X:24,568,551...24,571,012
Ensembl chr X:21,089,122...21,109,488
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Ivd
isovaleryl-CoA dehydrogenase
ISO
SMPDB
SMP:00138
NCBI chr 3:126,305,584...126,326,016
Ensembl chr 3:105,851,683...105,872,575
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Mccc1
methylcrotonyl-CoA carboxylase subunit 1
ISO
SMPDB
SMP:00138
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:00138
NCBI chr 2:33,039,044...33,110,092
Ensembl chr 2:31,304,932...31,375,972
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Mcee
methylmalonyl CoA epimerase
ISO
SMPDB
SMP:00138
NCBI chr 1:127,376,311...127,399,551
Ensembl chr 1:117,964,576...117,988,484
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Mmut
methylmalonyl-CoA mutase
ISO
SMPDB
SMP:00138
NCBI chr 9:27,425,935...27,454,202
Ensembl chr 9:19,928,727...19,957,046
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Oxct1
3-oxoacid CoA transferase 1
ISO
SMPDB
SMP:00138
NCBI chr 2:54,963,964...55,112,303
Ensembl chr 2:53,236,368...53,384,714
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Pcca
propionyl-CoA carboxylase subunit alpha
ISO
SMPDB
SMP:00138
NCBI chr15:106,034,586...106,374,908
Ensembl chr15:99,627,982...99,968,266
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Pccb
propionyl-CoA carboxylase subunit beta
ISO
SMPDB
SMP:00138
NCBI chr 8:110,470,197...110,520,222
Ensembl chr 8:101,590,737...101,641,234
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