full name
enoyl-CoA hydratase, short chain, 1, mitochondrial

synonyms
SCEH, ECHS1, ENSG00000127884

HGNC
HGNC:3151

Entrez Gene
Gene:1892

ENSEMBL
ENSG00000127884

location
chr: 10
start: 135175987
stop: 135186908
strand: -

OMIM
OMIM:602292: ECHS1
OMIM:616277: Mitochondrial short-chain enoyl-CoA hydratase 1 deficiency

transcripts
NM_004092.3

pathways
Beta oxidation of butanoyl-CoA to acetyl-CoA [NCBI:1270023] [REACTOME:R-HSA-77352]
Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA [NCBI:1270020] [REACTOME:R-HSA-77346]
Beta oxidation of hexanoyl-CoA to butanoyl-CoA [NCBI:1270022] [REACTOME:R-HSA-77350]
Beta oxidation of lauroyl-CoA to decanoyl-CoA-CoA [NCBI:1270019] [REACTOME:R-HSA-77310]
Beta oxidation of octanoyl-CoA to hexanoyl-CoA [NCBI:1270021] [REACTOME:R-HSA-77348]
Butanoate metabolism [NCBI:83007] [KEGG:hsa00650]
Carbon metabolism [NCBI:814926] [KEGG:hsa01200]
Fatty Acid Beta Oxidation [NCBI:198865] [WikiPathways:WP143]
Fatty Acid Biosynthesis [NCBI:198873] [WikiPathways:WP357]
Fatty acid biosynthesis, elongation, mitochondria [NCBI:413380] [KEGG:hsa_M00085]
Fatty acid degradation [NCBI:82935] [KEGG:hsa00071] [PUBMED:869535]
Fatty acid elongation [NCBI:82934] [KEGG:hsa00062] [PUBMED:16564093] [PUBMED:4387390] [PUBMED:8190743] [PUBMED:16866954] [PUBMED:19649180] [PUBMED:7150615] [PUBMED:16765910] [PUBMED:17719534]
Fatty acid metabolism [NCBI:868084] [KEGG:hsa01212]
Fatty acid, triacylglycerol, and ketone body metabolism [NCBI:1270010] [REACTOME:R-HSA-535734]
Lysine degradation [NCBI:82956] [KEGG:hsa00310] [PUBMED:1682209] [PUBMED:11802770]
Lysine degradation, lysine => saccharopine => acetoacetyl-CoA [NCBI:413352] [KEGG:hsa_M00032]
Malonate semialdehyde pathway, propanoyl-CoA => acetyl-CoA [NCBI:413478] [KEGG:hsa_M00013]
Metabolic pathways [NCBI:132956] [KEGG:hsa01100]
Metabolism [NCBI:1269956] [REACTOME:R-HSA-1430728]
Metabolism of lipids and lipoproteins [NCBI:1270001] [REACTOME:R-HSA-556833]
Mitochondrial Fatty Acid Beta-Oxidation [NCBI:1270015] [REACTOME:R-HSA-77289] [PUBMED:10527676] [PUBMED:11826276] [PUBMED:7951672] [PUBMED:1431593]
Propanoate metabolism [NCBI:83004] [KEGG:hsa00640]
Tryptophan metabolism [NCBI:82964] [KEGG:hsa00380] [NCBI:198850] [WikiPathways:WP465] [PUBMED:15749753]
Valine, leucine and isoleucine degradation [NCBI:82952] [KEGG:hsa00280]
beta-Alanine metabolism [NCBI:82966] [KEGG:hsa00410]
beta-Oxidation [NCBI:413381] [KEGG:hsa_M00087]
fatty acid beta-oxidation [NCBI:142421] [BIOCYC:HUMAN_FAO-PWY]
glutaryl-CoA degradation [NCBI:142409] [BIOCYC:HUMAN_PWY-5177] [PUBMED:8436125] [PUBMED:11705404]
isoleucine degradation [NCBI:142358] [BIOCYC:HUMAN_ILEUDEG-PWY] [PUBMED:4150713] [PUBMED:773366]
mitochondrial fatty acid beta-oxidation of saturated fatty acids [NCBI:1270016] [REACTOME:R-HSA-77286]
valine degradation [NCBI:142387] [BIOCYC:HUMAN_VALDEG-PWY] [PUBMED:10745006] [PUBMED:3827880] [PUBMED:6933702]