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
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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GFIT
Motif
Pfam:
Acyl-CoA_dh_N
Acyl-CoA_dh_1
Acyl-CoA_dh_M
Acyl-CoA_dh_2
Motif
Other DBs
NCBI-GeneID:
3712
NCBI-ProteinID:
NP_002216
OMIM:
607036
HGNC:
6186
Ensembl:
ENSG00000128928
UniProt:
P26440
A0A0A0MT83
Structure
PDB
PDBj
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All DBs
Position
15:40405795..40435947
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AA seq
423 aa
AA seq
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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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