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
Class
Metabolism; Overview; Carbon metabolism [PATH:hsa01200]
Metabolism; Overview; Fatty acid metabolism [PATH:hsa01212]
Metabolism; Carbohydrate metabolism; Propanoate metabolism [PATH:hsa00640]
Metabolism; Carbohydrate metabolism; Butanoate metabolism [PATH:hsa00650]
Metabolism; Lipid metabolism; Fatty acid elongation [PATH:hsa00062]
Metabolism; Lipid metabolism; Fatty acid degradation [PATH:hsa00071]
Metabolism; Lipid metabolism; Biosynthesis of unsaturated fatty acids [PATH:hsa01040]
Metabolism; Amino acid metabolism; Valine, leucine and isoleucine degradation [PATH:hsa00280]
Metabolism; Amino acid metabolism; Lysine degradation [PATH:hsa00310]
Metabolism; Amino acid metabolism; Tryptophan metabolism [PATH:hsa00380]
Metabolism; Metabolism of other amino acids; beta-Alanine metabolism [PATH:hsa00410]
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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