KEGG   Homo sapiens (human): 3712
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)
SSDB
Motif
Pfam: Acyl-CoA_dh_1 Acyl-CoA_dh_N Acyl-CoA_dh_M Acyl-CoA_dh_2
Other DBs
NCBI-GeneID: 3712
NCBI-ProteinID: NP_002216
OMIM: 607036
HGNC: 6186
Ensembl: ENSG00000128928
Vega: OTTHUMG00000129984
Pharos: P26440(Tbio)
UniProt: P26440 A0A0A0MT83
Structure
LinkDB
Position
15:40405795..40435947
AA seq 423 aa
MATATRLLGWRVASWRLRPPLAGFVSQRAHSLLPVDDAINGLSEEQRQLRQTMAKFLQEH
LAPKAQEIDRSNEFKNLREFWKQLGNLGVLGITAPVQYGGSGLGYLEHVLVMEEISRASG
AVGLSYGAHSNLCINQLVRNGNEAQKEKYLPKLISGEYIGALAMSEPNAGSDVVSMKLKA
EKKGNHYILNGNKFWITNGPDADVLIVYAKTDLAAVPASRGITAFIVEKGMPGFSTSKKL
DKLGMRGSNTCELIFEDCKIPAANILGHENKGVYVLMSGLDLERLVLAGGPLGLMQAVLD
HTIPYLHVREAFGQKIGHFQLMQGKMADMYTRLMACRQYVYNVAKACDEGHCTAKDCAGV
ILYSAECATQVALDGIQCFGGNGYINDFPMGRFLRDAKLYEIGAGTSEVRRLVIGRAFNA
DFH
NT seq 1272 nt   +upstreamnt  +downstreamnt
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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