Homo sapiens (human): 3712
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Entry
3712 CDS
T01001
Symbol
IVD, ACAD2, IVDH
Name
(RefSeq) isovaleryl-CoA dehydrogenase
KO
K00253
isovaleryl-CoA dehydrogenase [EC:
1.3.8.4
]
Organism
hsa
Homo sapiens (human)
Pathway
hsa00280
Valine, leucine and isoleucine degradation
hsa01100
Metabolic pathways
Module
hsa_M00036
Leucine degradation, leucine => acetoacetate + acetyl-CoA
Network
nt06024
Valine, leucine and isoleucine degradation
Element
N00833
HMGCL deficiency in leucine degradation
N00834
AUH deficiency in leucine degradation
N00835
MCCC1 deficiency in leucine degradation
N00836
MCCC2 deficiency in leucine degradation
N00837
IVD deficiency in leucine degradation
N00851
Leucine degradation
Disease
H00173
Isovaleric acidemia
H01400
Secondary hyperammonemia
Brite
KEGG Orthology (KO) [BR:
hsa00001
]
09100 Metabolism
09105 Amino acid metabolism
00280 Valine, leucine and isoleucine degradation
3712 (IVD)
Enzymes [BR:
hsa01000
]
1. Oxidoreductases
1.3 Acting on the CH-CH group of donors
1.3.8 With a flavin as acceptor
1.3.8.4 isovaleryl-CoA dehydrogenase
3712 (IVD)
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Ortholog
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GFIT
Motif
Pfam:
Acyl-CoA_dh_1
Acyl-CoA_dh_N
Acyl-CoA_dh_M
Acyl-CoA_dh_2
Motif
Other DBs
NCBI-GeneID:
3712
NCBI-ProteinID:
NP_002216
OMIM:
607036
HGNC:
6186
Ensembl:
ENSG00000128928
Vega:
OTTHUMG00000129984
Pharos:
P26440
(Tbio)
UniProt:
P26440
A0A0A0MT83
Structure
PDB
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All DBs
Position
15:40405795..40435947
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AA seq
423 aa
AA seq
DB search
MATATRLLGWRVASWRLRPPLAGFVSQRAHSLLPVDDAINGLSEEQRQLRQTMAKFLQEH
LAPKAQEIDRSNEFKNLREFWKQLGNLGVLGITAPVQYGGSGLGYLEHVLVMEEISRASG
AVGLSYGAHSNLCINQLVRNGNEAQKEKYLPKLISGEYIGALAMSEPNAGSDVVSMKLKA
EKKGNHYILNGNKFWITNGPDADVLIVYAKTDLAAVPASRGITAFIVEKGMPGFSTSKKL
DKLGMRGSNTCELIFEDCKIPAANILGHENKGVYVLMSGLDLERLVLAGGPLGLMQAVLD
HTIPYLHVREAFGQKIGHFQLMQGKMADMYTRLMACRQYVYNVAKACDEGHCTAKDCAGV
ILYSAECATQVALDGIQCFGGNGYINDFPMGRFLRDAKLYEIGAGTSEVRRLVIGRAFNA
DFH
NT seq
1272 nt
NT seq
+upstream
nt +downstream
nt
atggcgactgcgactcggctgctggggtggcgtgtggcgagctggaggctgcggccgccg
cttgccggcttcgtttcccagcgggcccactcgcttttgcccgtggacgatgcaatcaat
gggctaagcgaggagcagaggcagcttcgtcagaccatggctaagttccttcaggagcac
ctggcccccaaggcccaggagatcgatcgcagcaatgagttcaagaacctgcgagaattt
tggaagcagctggggaacctgggcgtattgggcatcacagcccctgttcagtatggcggc
tccggcctgggctacctggagcatgtgctggtgatggaggagatatcccgagcttccgga
gcagtggggctcagttacggtgcccactccaacctctgcatcaaccagcttgtacgcaat
gggaatgaggcccagaaagagaagtatctcccgaagctgatcagtggtgagtacatcgga
gccctggccatgagtgagcccaatgcaggctctgatgttgtctctatgaagctcaaagcg
gaaaagaaaggaaatcactacatcctgaatggcaacaagttctggatcactaatggccct
gatgctgacgtcctgattgtctatgccaagacagatctggctgctgtgccagcttctcgg
ggcatcacagccttcattgtggagaagggtatgcctggctttagcacctctaagaagctg
gacaagctggggatgaggggctctaacacctgtgagctaatctttgaagactgcaagatt
cctgctgccaacatcctgggccatgagaataagggtgtctacgtgctgatgagtgggctg
gacctggagcggctggtgctggccggggggcctcttgggctcatgcaagcggtcctggac
cacaccattccctacctgcacgtgagggaagcctttggccagaagatcggccacttccag
ttgatgcaggggaagatggctgacatgtacacccgcctcatggcgtgtcggcagtatgtc
tacaatgtcgccaaggcctgcgatgagggccattgcactgctaaggactgtgcaggtgtg
attctttactcagctgagtgtgccacacaggtagccctggacggcattcagtgttttggt
ggcaatggctacatcaatgactttcccatgggccgctttcttcgagatgccaagctgtat
gagataggggctgggaccagcgaggtgaggcggctggtcatcggcagagccttcaatgca
gactttcactag
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