KEGG   Homo sapiens (human): 3030Help
Entry
3030              CDS       T01001                                 

Gene name
HADHA, ECHA, GBP, HADH, LCEH, LCHAD, MTPA, TP-ALPHA
Definition
hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit (EC:4.2.1.17 1.1.1.211)
Orthology
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
Fatty acid elongation
Fatty acid degradation
Valine, leucine and isoleucine degradation
Lysine degradation
Tryptophan metabolism
beta-Alanine metabolism
Propanoate metabolism
Butanoate metabolism
Biosynthesis of unsaturated fatty acids
Metabolic pathways
Carbon metabolism
Fatty acid metabolism
Module
Malonate semialdehyde pathway, propanoyl-CoA => Acetyl-CoA
Lysine degradation, lysine => saccharopine => acetoacetyl-CoA
Fatty acid biosynthesis, elongation, mitochondria
beta-Oxidation
Disease
H00525  
Disorders of fatty-acid oxidation
H01352  
Mitochondrial trifunctional protein (TFP) deficiency
Brite
KEGG Orthology (KO) [BR:hsa00001]
 Metabolism
  Overview
   01200 Carbon metabolism
    3030 (HADHA)
   01212 Fatty acid metabolism
    3030 (HADHA)
  Carbohydrate metabolism
   00640 Propanoate metabolism
    3030 (HADHA)
   00650 Butanoate metabolism
    3030 (HADHA)
  Lipid metabolism
   00062 Fatty acid elongation
    3030 (HADHA)
   00071 Fatty acid degradation
    3030 (HADHA)
   01040 Biosynthesis of unsaturated fatty acids
    3030 (HADHA)
  Amino acid metabolism
   00280 Valine, leucine and isoleucine degradation
    3030 (HADHA)
   00310 Lysine degradation
    3030 (HADHA)
   00380 Tryptophan metabolism
    3030 (HADHA)
  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)
BRITE hierarchy
SSDB OrthologParalogGFIT
Motif Motif
Other DBs
NCBI-GI: 
NCBI-GeneID: 
OMIM: 
HGNC: 
HPRD: 
Ensembl: 
Vega: 
UniProt: 
Position
2p23
AA seq 763 aa AA seqDB search
MVACRAIGILSRFSAFRILRSRGYICRNFTGSSALLTRTHINYGVKGDVAVVRINSPNSK
VNTLSKELHSEFSEVMNEIWASDQIRSAVLISSKPGCFIAGADINMLAACKTLQEVTQLS
QEAQRIVEKLEKSTKPIVAAINGSCLGGGLEVAISCQYRIATKDRKTVLGTPEVLLGALP
GAGGTQRLPKMVGVPAALDMMLTGRSIRADRAKKMGLVDQLVEPLGPGLKPPEERTIEYL
EEVAITFAKGLADKKISPKRDKGLVEKLTAYAMTIPFVRQQVYKKVEEKVRKQTKGLYPA
PLKIIDVVKTGIEQGSDAGYLCESQKFGELVMTKESKALMGLYHGQVLCKKNKFGAPQKD
VKHLAILGAGLMGAGIAQVSVDKGLKTILKDATLTALDRGQQQVFKGLNDKVKKKALTSF
ERDSIFSNLTGQLDYQGFEKADMVIEAVFEDLSLKHRVLKEVEAVIPDHCIFASNTSALP
ISEIAAVSKRPEKVIGMHYFSPVDKMQLLEIITTEKTSKDTSASAVAVGLKQGKVIIVVK
DGPGFYTTRCLAPMMSEVIRILQEGVDPKKLDSLTTSFGFPVGAATLVDEVGVDVAKHVA
EDLGKVFGERFGGGNPELLTQMVSKGFLGRKSGKGFYIYQEGVKRKDLNSDMDSILASLK
LPPKSEVSSDEDIQFRLVTRFVNEAVMCLQEGILATPAEGDIGAVFGLGFPPCLGGPFRF
VDLYGAQKIVDRLKKYEAAYGKQFTPCQLLADHANSPNKKFYQ
NT seq 2292 nt NT seq  +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

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