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
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_N Acyl-CoA_dh_1 Acyl-CoA_dh_M Acyl-CoA_dh_2
Other DBs
NCBI-GeneID: 3712
NCBI-ProteinID: NP_002216
OMIM: 607036
HGNC: 6186
Ensembl: ENSG00000128928
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

KEGG   Homo sapiens (human): 34
Entry
34                CDS       T01001                                 
Symbol
ACADM, ACAD1, MCAD, MCADH
Name
(RefSeq) acyl-CoA dehydrogenase medium chain
  KO
K00249  acyl-CoA dehydrogenase [EC:1.3.8.7]
Organism
hsa  Homo sapiens (human)
Pathway
hsa00071  Fatty acid degradation
hsa00280  Valine, leucine and isoleucine degradation
hsa01100  Metabolic pathways
hsa01212  Fatty acid metabolism
hsa03320  PPAR signaling pathway
hsa04936  Alcoholic liver disease
Module
hsa_M00036  Leucine degradation, leucine => acetoacetate + acetyl-CoA
hsa_M00087  beta-Oxidation
Network
nt06020  beta-Oxidation in mitochondria
nt06024  Valine, leucine and isoleucine degradation
  Element
N00804  beta-Oxidation
N00851  Leucine degradation
N00852  Valine degradation
N00856  Isoleucine degradation
Disease
H00488  MCAD deficiency
H00525  Disorders of mitochondrial fatty-acid oxidation
H01400  Secondary hyperammonemia
Brite
KEGG Orthology (KO) [BR:hsa00001]
 09100 Metabolism
  09103 Lipid metabolism
   00071 Fatty acid degradation
    34 (ACADM)
  09105 Amino acid metabolism
   00280 Valine, leucine and isoleucine degradation
    34 (ACADM)
 09150 Organismal Systems
  09152 Endocrine system
   03320 PPAR signaling pathway
    34 (ACADM)
 09160 Human Diseases
  09167 Endocrine and metabolic disease
   04936 Alcoholic liver disease
    34 (ACADM)
Enzymes [BR:hsa01000]
 1. Oxidoreductases
  1.3  Acting on the CH-CH group of donors
   1.3.8  With a flavin as acceptor
    1.3.8.7  medium-chain acyl-CoA dehydrogenase
     34 (ACADM)
SSDB
Motif
Pfam: Acyl-CoA_dh_1 Acyl-CoA_dh_N Acyl-CoA_dh_M Acyl-CoA_dh_2 HpaB_N ACOX_C_alpha1
Other DBs
NCBI-GeneID: 34
NCBI-ProteinID: NP_000007
OMIM: 607008
HGNC: 89
Ensembl: ENSG00000117054
UniProt: P11310 A0A0S2Z366
Structure
LinkDB
Position
1:75724709..75763679
AA seq 421 aa
MAAGFGRCCRVLRSISRFHWRSQHTKANRQREPGLGFSFEFTEQQKEFQATARKFAREEI
IPVAAEYDKTGEYPVPLIRRAWELGLMNTHIPENCGGLGLGTFDACLISEELAYGCTGVQ
TAIEGNSLGQMPIIIAGNDQQKKKYLGRMTEEPLMCAYCVTEPGAGSDVAGIKTKAEKKG
DEYIINGQKMWITNGGKANWYFLLARSDPDPKAPANKAFTGFIVEADTPGIQIGRKELNM
GQRCSDTRGIVFEDVKVPKENVLIGDGAGFKVAMGAFDKTRPVVAAGAVGLAQRALDEAT
KYALERKTFGKLLVEHQAISFMLAEMAMKVELARMSYQRAAWEVDSGRRNTYYASIAKAF
AGDIANQLATDAVQILGGNGFNTEYPVEKLMRDAKIYQIYEGTSQIQRLIVAREHIDKYK
N
NT seq 1266 nt   +upstreamnt  +downstreamnt
atggcagcggggttcgggcgatgctgcagggtcctgagaagtatttctcgttttcattgg
agatcacagcatacaaaagccaatcgacaacgtgaaccaggattaggatttagttttgag
ttcaccgaacagcagaaagaatttcaagctactgctcgtaaatttgccagagaggaaatc
atcccagtggctgcagaatatgataaaactggtgaatatccagtccccctaattagaaga
gcctgggaacttggtttaatgaacacacacattccagagaactgtggaggtcttggactt
ggaacttttgatgcttgtttaattagtgaagaattggcttatggatgtacaggggttcag
actgctattgaaggaaattctttggggcaaatgcctattattattgctggaaatgatcaa
caaaagaagaagtatttggggagaatgactgaggagccattgatgtgtgcttattgtgta
acagaacctggagcaggctctgatgtagctggtataaagaccaaagcagaaaagaaagga
gatgagtatattattaatggtcagaagatgtggataaccaacggaggaaaagctaattgg
tattttttattggcacgttctgatccagatcctaaagctcctgctaataaagcctttact
ggattcattgtggaagcagataccccaggaattcagattgggagaaaggaattaaacatg
ggccagcgatgttcagatactagaggaattgtcttcgaagatgtgaaagtgcctaaagaa
aatgttttaattggtgacggagctggtttcaaagttgcaatgggagcttttgataaaacc
agacctgtagtagctgctggtgctgttggattagcacaaagagctttggatgaagctacc
aagtatgccctggaaaggaaaactttcggaaagctacttgtagagcaccaagcaatatca
tttatgctggctgaaatggcaatgaaagttgaactagctagaatgagttaccagagagca
gcttgggaggttgattctggtcgtcgaaatacctattatgcttctattgcaaaggcattt
gctggagatattgcaaatcagttagctactgatgctgtgcagatacttggaggcaatgga
tttaatacagaatatcctgtagaaaaactaatgagggatgccaaaatctatcagatttat
gaaggtacttcacaaattcaaagacttattgtagcccgtgaacacattgacaagtacaaa
aattaa

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