KEGG   Homo sapiens (human): 3030
Entry
3030              CDS       T01001                                 
Symbol
HADHA, ECHA, GBP, HADH, LCEH, LCHAD, MTPA, TP-ALPHA
Name
(RefSeq) hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha
  KO
K07515  enoyl-CoA hydratase / long-chain 3-hydroxyacyl-CoA dehydrogenase [EC:4.2.1.17 1.1.1.211]
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
hsa00410  beta-Alanine metabolism
hsa00640  Propanoate 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
N00804  beta-Oxidation
N00852  Valine degradation
N00856  Isoleucine degradation
Disease
H00489  LCHAD deficiency
H00525  Disorders of mitochondrial fatty-acid oxidation
H01352  Mitochondrial trifunctional protein deficiency
Brite
KEGG Orthology (KO) [BR:hsa00001]
 09100 Metabolism
  09101 Carbohydrate metabolism
   00640 Propanoate metabolism
    3030 (HADHA)
   00650 Butanoate metabolism
    3030 (HADHA)
  09103 Lipid metabolism
   00062 Fatty acid elongation
    3030 (HADHA)
   00071 Fatty acid degradation
    3030 (HADHA)
  09105 Amino acid metabolism
   00280 Valine, leucine and isoleucine degradation
    3030 (HADHA)
   00310 Lysine degradation
    3030 (HADHA)
   00380 Tryptophan metabolism
    3030 (HADHA)
  09106 Metabolism of other amino acids
   00410 beta-Alanine metabolism
    3030 (HADHA)
Enzymes [BR:hsa01000]
 1. Oxidoreductases
  1.1  Acting on the CH-OH group of donors
   1.1.1  With NAD+ or NADP+ as acceptor
    1.1.1.211  long-chain-3-hydroxyacyl-CoA dehydrogenase
     3030 (HADHA)
 4. Lyases
  4.2  Carbon-oxygen lyases
   4.2.1  Hydro-lyases
    4.2.1.17  enoyl-CoA hydratase
     3030 (HADHA)
SSDB
Motif
Pfam: 3HCDH_N ECH_1 3HCDH ECH_2
Other DBs
NCBI-GeneID: 3030
NCBI-ProteinID: NP_000173
OMIM: 600890
HGNC: 4801
Ensembl: ENSG00000084754
Pharos: P40939(Tbio)
UniProt: P40939 E9KL44
Structure
LinkDB
Position
2:complement(26190635..26244632)
AA seq 763 aa
MVACRAIGILSRFSAFRILRSRGYICRNFTGSSALLTRTHINYGVKGDVAVVRINSPNSK
VNTLSKELHSEFSEVMNEIWASDQIRSAVLISSKPGCFIAGADINMLAACKTLQEVTQLS
QEAQRIVEKLEKSTKPIVAAINGSCLGGGLEVAISCQYRIATKDRKTVLGTPEVLLGALP
GAGGTQRLPKMVGVPAALDMMLTGRSIRADRAKKMGLVDQLVEPLGPGLKPPEERTIEYL
EEVAITFAKGLADKKISPKRDKGLVEKLTAYAMTIPFVRQQVYKKVEEKVRKQTKGLYPA
PLKIIDVVKTGIEQGSDAGYLCESQKFGELVMTKESKALMGLYHGQVLCKKNKFGAPQKD
VKHLAILGAGLMGAGIAQVSVDKGLKTILKDATLTALDRGQQQVFKGLNDKVKKKALTSF
ERDSIFSNLTGQLDYQGFEKADMVIEAVFEDLSLKHRVLKEVEAVIPDHCIFASNTSALP
ISEIAAVSKRPEKVIGMHYFSPVDKMQLLEIITTEKTSKDTSASAVAVGLKQGKVIIVVK
DGPGFYTTRCLAPMMSEVIRILQEGVDPKKLDSLTTSFGFPVGAATLVDEVGVDVAKHVA
EDLGKVFGERFGGGNPELLTQMVSKGFLGRKSGKGFYIYQEGVKRKDLNSDMDSILASLK
LPPKSEVSSDEDIQFRLVTRFVNEAVMCLQEGILATPAEGDIGAVFGLGFPPCLGGPFRF
VDLYGAQKIVDRLKKYEAAYGKQFTPCQLLADHANSPNKKFYQ
NT seq 2292 nt   +upstreamnt  +downstreamnt
atggtggcctgccgggcgattggcatcctcagccgcttttctgccttcaggatcctccgc
tcccgaggttatatatgccgcaattttacagggtcttctgctttgctgaccagaacccat
attaactatggagtcaaaggggatgtggcagttgttcgaattaactctcccaattcaaag
gtaaatacactgagtaaagagctacattcagagttctcagaagttatgaatgaaatctgg
gctagtgatcaaatcagaagtgccgtccttatctcatcaaagccaggctgctttattgca
ggtgctgatatcaacatgttagccgcttgcaagacccttcaagaagtaacacagctatca
caagaagcacagagaatagttgagaaacttgaaaagtccacaaagcctattgtggctgcc
atcaatggatcctgcctgggaggaggacttgaggttgccatttcatgccaatacagaata
gcaacaaaagacagaaaaacagtattaggtacccctgaagttttgctgggggccttacca
ggagcaggaggcacacaaaggctgcccaaaatggtgggtgtgcctgctgctttggacatg
atgctgactggtagaagcattcgtgcagacagggcaaagaaaatgggactggttgaccaa
ctggtggaacccctgggaccaggactaaaacctccagaggaacggacaatagaataccta
gaagaagttgcaattacttttgccaaaggactagctgataagaagatctctccaaagaga
gacaagggattggtggaaaaattgacagcgtatgccatgactattccatttgtcaggcaa
caggtttacaaaaaagtggaagaaaaagtgcgaaagcagactaaaggcctttatcctgca
cctctgaaaataattgatgtggtaaagactggaattgagcaagggagtgatgccggttat
ctctgtgaatctcagaaatttggagagcttgtaatgaccaaagaatcaaaggccttgatg
ggactctaccatggtcaggtcctgtgcaagaagaataaatttggagctccacagaaggat
gttaagcatctggctattcttggtgcagggctgatgggagcaggcatcgcccaagtctcc
gtggataaggggctaaagactatacttaaagatgccaccctcactgcgctagaccgagga
cagcaacaagtgttcaaaggattgaatgacaaagtgaagaagaaagctctaacatcattt
gaaagggattccatcttcagcaacttgactgggcagcttgattaccaaggttttgaaaag
gccgacatggtgattgaagctgtgtttgaggaccttagtcttaagcacagagtgctaaag
gaagtagaagcggtgattccagatcactgtatctttgccagtaacacatctgctctccca
atcagtgaaatcgctgctgtcagcaaaagacctgagaaggtgattggcatgcactacttc
tctcccgtggacaagatgcagctgctggagattatcacgaccgagaaaacttccaaagac
accagtgcttcagctgtagcagttggtctcaagcaggggaaggtcatcattgtggttaag
gatggacctggcttctatactaccaggtgtcttgcgcccatgatgtctgaagtcatccga
atcctccaggaaggagttgacccgaagaagctggattccctgaccacaagctttggcttt
cctgtgggtgccgccacactggtggatgaagttggtgtggatgtagcgaaacatgtggcg
gaagatctgggcaaagtctttggggagcggtttggaggtggaaacccagaactgctgaca
cagatggtgtccaagggcttcctaggtcgtaaatctgggaagggcttttacatctatcag
gagggtgtgaagaggaaggatttgaattctgacatggatagtattttagcgagtctgaag
ctgcctcctaagtctgaagtctcatcagacgaagacatccagttccgcctggtgacaaga
tttgtgaatgaggcagtcatgtgcctgcaagaggggatcttggccacacctgcagaggga
gacatcggagccgtctttgggcttggcttcccgccttgtctgggagggcctttccgcttt
gtggatctgtatggcgcccagaagatagtggaccggctcaagaaatatgaagctgcctat
ggaaaacagttcaccccatgccagctgctagctgaccatgctaacagccctaacaagaag
ttctaccagtga

DBGET integrated database retrieval system