KEGG   Homo sapiens (human): 1962
Entry
1962              CDS       T01001                                 

Gene name
EHHADH, ECHD, FRTS3, L-PBE, LBFP, LBP, MFE1, PBFE
Definition
(RefSeq) enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase
  KO
K07514  enoyl-CoA hydratase / 3-hydroxyacyl-CoA dehydrogenase / 3,2-trans-enoyl-CoA isomerase [EC:4.2.1.17 1.1.1.35 5.3.3.8]
Organism
hsa  Homo sapiens (human)
Pathway
hsa00071  Fatty acid degradation
hsa00280  Valine, leucine and isoleucine degradation
hsa00310  Lysine degradation
hsa00380  Tryptophan metabolism
hsa00410  beta-Alanine metabolism
hsa00640  Propanoate metabolism
hsa00650  Butanoate metabolism
hsa01100  Metabolic pathways
hsa01200  Carbon metabolism
hsa01212  Fatty acid metabolism
hsa03320  PPAR signaling pathway
hsa04146  Peroxisome
Module
hsa_M00032  Lysine degradation, lysine => saccharopine => acetoacetyl-CoA
hsa_M00087  beta-Oxidation
Disease
H01198  Fanconi renotubular syndrome
Brite
KEGG Orthology (KO) [BR:hsa00001]
 09100 Metabolism
  09101 Carbohydrate metabolism
   00640 Propanoate metabolism
    1962 (EHHADH)
   00650 Butanoate metabolism
    1962 (EHHADH)
  09103 Lipid metabolism
   00071 Fatty acid degradation
    1962 (EHHADH)
  09105 Amino acid metabolism
   00280 Valine, leucine and isoleucine degradation
    1962 (EHHADH)
   00310 Lysine degradation
    1962 (EHHADH)
   00380 Tryptophan metabolism
    1962 (EHHADH)
  09106 Metabolism of other amino acids
   00410 beta-Alanine metabolism
    1962 (EHHADH)
 09140 Cellular Processes
  09141 Transport and catabolism
   04146 Peroxisome
    1962 (EHHADH)
 09150 Organismal Systems
  09152 Endocrine system
   03320 PPAR signaling pathway
    1962 (EHHADH)
Enzymes [BR:hsa01000]
 1. Oxidoreductases
  1.1  Acting on the CH-OH group of donors
   1.1.1  With NAD+ or NADP+ as acceptor
    1.1.1.35  3-hydroxyacyl-CoA dehydrogenase
     1962 (EHHADH)
 4. Lyases
  4.2  Carbon-oxygen lyases
   4.2.1  Hydro-lyases
    4.2.1.17  enoyl-CoA hydratase
     1962 (EHHADH)
 5. Isomerases
  5.3  Intramolecular oxidoreductases
   5.3.3  Transposing C=C bonds
    5.3.3.8  Delta3-Delta2-enoyl-CoA isomerase
     1962 (EHHADH)
SSDB
Motif
Pfam: 3HCDH_N ECH_1 3HCDH ECH_2 NAD_binding_2 UDPG_MGDP_dh_N Peptidase_S49 TrkA_N 2-Hacid_dh_C
Other DBs
NCBI-GeneID: 1962
NCBI-ProteinID: NP_001957
OMIM: 607037
HGNC: 3247
Ensembl: ENSG00000113790
Vega: OTTHUMG00000156698
Pharos: Q08426(Tbio)
UniProt: Q08426
LinkDB
Position
3q27.2
AA seq 723 aa
MAEYTRLHNALALIRLRNPPVNAISTTLLRDIKEGLQKAVIDHTIKAIVICGAEGKFSAG
ADIRGFSAPRTFGLTLGHVVDEIQRNEKPVVAAIQGMAFGGGLELALGCHYRIAHAEAQV
GLPEVTLGLLPGARGTQLLPRLTGVPAALDLITSGRRILADEALKLGILDKVVNSDPVEE
AIRFAQRVSDQPLESRRLCNKPIQSLPNMDSIFSEALLKMRRQHPGCLAQEACVRAVQAA
VQYPYEVGIKKEEELFLYLLQSGQARALQYAFFAERKANKWSTPSGASWKTASARPVSSV
GVVGLGTMGRGIVISFARARIPVIAVDSDKNQLATANKMITSVLEKEASKMQQSGHPWSG
PKPRLTSSVKELGGVDLVIEAVFEEMSLKKQVFAELSAVCKPEAFLCTNTSALDVDEIAS
STDRPHLVIGTHFFSPAHVMKLLEVIPSQYSSPTTIATVMNLSKKIKKIGVVVGNCFGFV
GNRMLNPYYNQAYFLLEEGSKPEEVDQVLEEFGFKMGPFRVSDLAGLDVGWKSRKGQGLT
GPTLLPGTPARKRGNRRYCPIPDVLCELGRFGQKTGKGWYQYDKPLGRIHKPDPWLSKFL
SRYRKTHHIEPRTISQDEILERCLYSLINEAFRILGEGIAASPEHIDVVYLHGYGWPRHK
GGPMFYASTVGLPTVLEKLQKYYRQNPDIPQLEPSDYLKKLASQGNPPLKEWQSLAGSPS
SKL
NT seq 2172 nt   +upstreamnt  +downstreamnt
atggccgagtatacgcggctgcacaacgccttggcgctaatccgcctccgaaacccgccg
gtcaacgcgatcagtacgactttactccgtgacataaaagaaggactacagaaagctgta
atagaccatacaataaaagccattgtgatttgtggagcagagggcaaattttctgcaggt
gctgatattcgtggcttcagtgctcctaggacatttggccttacactgggacatgtagta
gatgaaatacagagaaatgagaagcccgtggtggcagcaatccaaggcatggctttcgga
gggggactagagctggccctgggctgtcactataggattgcccacgcagaggctcaagtt
ggcttaccagaagttacactgggacttctccctggtgcaagaggaacccagcttctcccc
agactcactggagttcctgctgcacttgacttaattacctcaggaagacgtattttagca
gatgaagcactcaagctgggcattctagataaagttgtaaactcagacccggttgaagaa
gcaatcagatttgctcagagagtttcagatcaacctctagaatcccgtagactctgcaac
aagccaattcagagcttgcccaacatggacagcatttttagtgaggccctcttgaagatg
cggaggcagcaccctgggtgtcttgcacaggaggcttgtgtccgtgcagtccaggctgct
gtgcagtatccctatgaagtgggcatcaagaaggaggaggagctgtttctatatcttttg
caatcagggcaggctagagccctgcaatatgctttcttcgctgaaaggaaagcaaataag
tggtcaactccctccggagcatcgtggaaaacagcatcagcgcggcctgtctcctcagtt
ggtgttgttggcttgggaacaatgggccgaggcattgtcatttcttttgcaagggccagg
attcctgtgattgctgtagactcggacaaaaaccagctagcaactgcaaacaagatgata
acctctgtcttggaaaaagaagcctccaaaatgcaacagagcggccacccttggtcagga
ccaaaacccaggttaacttcatctgtgaaggagcttggtggtgtagatttagtcattgaa
gcagtatttgaggaaatgagcctgaagaagcaggtctttgctgaactctcagctgtgtgc
aaaccagaagcatttttgtgcactaatacttcagccctggatgttgatgagattgcttct
tccactgatcgtcctcacttggtcattggcacccacttcttttcgccagctcatgtcatg
aagttgttagaggttattcccagccaatactcttcccccactaccattgccactgttatg
aacttatcaaaaaagattaaaaagattggagtcgttgtaggcaactgttttggatttgtg
gggaatcgaatgttgaatccttactacaatcaggcatatttcttgttagaagaaggcagc
aaaccagaggaggtagatcaggtgctggaagagtttggttttaaaatgggaccttttaga
gtgtctgatcttgctgggttggatgtgggctggaaatctagaaaggggcaaggtcttact
ggacctacattgcttccaggaactcctgcccgaaaaaggggtaataggaggtactgccca
attcctgatgtgctctgtgaattaggacgatttggccagaagacaggtaagggttggtat
caatatgacaagccattgggtaggattcacaaacctgatccctggctttccaaattccta
tcacggtatagaaaaacccatcacattgaaccacgtaccattagccaggatgagatcctt
gaacgctgcttatattcacttatcaatgaagcattccgtatcttgggagaagggatagct
gctagcccagagcacattgatgttgtctatttacatggatatggatggccaaggcacaag
ggcgggcccatgttctatgcttccacagttgggttgcccacagttctagagaaattgcag
aaatattacaggcagaaccctgatattccccaactggagccaagtgactatctaaaaaaa
ctggcttctcagggaaaccctcccctgaaagaatggcaaagcttggcaggctcccctagc
agtaaattgtga

KEGG   Homo sapiens (human): 3033
Entry
3033              CDS       T01001                                 

Gene name
HADH, HAD, HADH1, HADHSC, HCDH, HHF4, MSCHAD, SCHAD
Definition
(RefSeq) hydroxyacyl-CoA dehydrogenase
  KO
K00022  3-hydroxyacyl-CoA dehydrogenase [EC:1.1.1.35]
Organism
hsa  Homo sapiens (human)
Pathway
hsa00062  Fatty acid elongation
hsa00071  Fatty acid degradation
hsa00280  Valine, leucine and isoleucine degradation
hsa00310  Lysine degradation
hsa00380  Tryptophan metabolism
hsa00650  Butanoate metabolism
hsa01100  Metabolic pathways
hsa01212  Fatty acid metabolism
Module
hsa_M00032  Lysine degradation, lysine => saccharopine => acetoacetyl-CoA
hsa_M00085  Fatty acid elongation in mitochondria
hsa_M00087  beta-Oxidation
Network
nt06020  beta-Oxidation in mitochondria
nt06024  Valine, leucine and isoleucine degradation
  Element
N00766  HADHB deficiency in beta-oxidation
N00767  HADH deficiency in beta-oxidation
N00804  beta-Oxidation
N00852  Valine degradation
N00853  ALDH6A1 deficiency in valine degradation
N00856  Isoleucine degradation
Disease
H00525  Disorders of mitochondrial fatty-acid oxidation
H01267  Familial hyperinsulinemic hypoglycemia
H01364  3-Hydroxyacyl-CoA dehydrogenase deficiency
Brite
KEGG Orthology (KO) [BR:hsa00001]
 09100 Metabolism
  09101 Carbohydrate metabolism
   00650 Butanoate metabolism
    3033 (HADH)
  09103 Lipid metabolism
   00062 Fatty acid elongation
    3033 (HADH)
   00071 Fatty acid degradation
    3033 (HADH)
  09105 Amino acid metabolism
   00280 Valine, leucine and isoleucine degradation
    3033 (HADH)
   00310 Lysine degradation
    3033 (HADH)
   00380 Tryptophan metabolism
    3033 (HADH)
Enzymes [BR:hsa01000]
 1. Oxidoreductases
  1.1  Acting on the CH-OH group of donors
   1.1.1  With NAD+ or NADP+ as acceptor
    1.1.1.35  3-hydroxyacyl-CoA dehydrogenase
     3033 (HADH)
SSDB
Motif
Pfam: 3HCDH_N 3HCDH Pyr_redox_2 UDPG_MGDP_dh_N Pyr_redox NAD_Gly3P_dh_N NAD_binding_2 DAO NAD_binding_7 AlaDh_PNT_C F420_oxidored GIDA 2-Hacid_dh_C NAD_binding_8 Sacchrp_dh_NADP XdhC_C FAD_oxidored
Other DBs
NCBI-GeneID: 3033
NCBI-ProteinID: NP_005318
OMIM: 601609
HGNC: 4799
Ensembl: ENSG00000138796
Vega: OTTHUMG00000131810
Pharos: Q16836(Tbio)
UniProt: Q16836 A0A140VK76
LinkDB
Structure
PDB: 

Position
4q25
AA seq 314 aa
MAFVTRQFMRSVSSSSTASASAKKIIVKHVTVIGGGLMGAGIAQVAAATGHTVVLVDQTE
DILAKSKKGIEESLRKVAKKKFAENLKAGDEFVEKTLSTIATSTDAASVVHSTDLVVEAI
VENLKVKNELFKRLDKFAAEHTIFASNTSSLQITSIANATTRQDRFAGLHFFNPVPVMKL
VEVIKTPMTSQKTFESLVDFSKALGKHPVSCKDTPGFIVNRLLVPYLMEAIRLYERGDAS
KEDIDTAMKLGAGYPMGPFELLDYVGLDTTKFIVDGWHEMDAENPLHQPSPSLNKLVAEN
KFGKKTGEGFYKYK
NT seq 945 nt   +upstreamnt  +downstreamnt
atggccttcgtcaccaggcagttcatgcgttccgtgtcctcctcgtccaccgcctcggcc
tcggccaagaagataatcgtcaagcacgtgacggtcatcggcggcgggctgatgggcgcc
ggcattgcccaggttgctgcagcaactggtcacacagtagtgttggtagaccagacagag
gacatcctggcaaaatccaaaaagggaattgaggaaagccttaggaaagtggcaaagaag
aagtttgcagaaaaccttaaggccggcgatgaatttgtggagaagaccctgagcaccata
gcgaccagcacggatgcagcctccgttgtccacagcacagacttggtggtggaagccatc
gtggagaatctgaaggtgaaaaacgagctcttcaaaaggctggacaagtttgctgctgaa
catacaatctttgccagcaacacttcctccttgcagattacaagcatagctaatgccacc
accagacaagaccgattcgctggcctccatttcttcaacccagtgcctgtcatgaaactt
gtggaggtcattaaaacaccaatgaccagccagaagacatttgaatctttggtagacttt
agcaaagccctaggaaagcatcctgtttcttgcaaggacactcctgggtttattgtgaac
cgcctcctggttccatacctcatggaagcaatcaggctgtatgaacgaggtgacgcatcc
aaagaagacattgacactgctatgaaattaggagccggttaccccatgggcccatttgag
cttctagattatgtcggactggatactacgaagttcatcgtggatgggtggcatgaaatg
gatgcagagaacccattacatcagcccagcccatccttaaataagctggtagcagagaac
aagttcggcaagaagactggagaaggattttacaaatacaagtga

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