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Official Full Name enoyl CoA hydratase, short chain, 1, mitochondrial
Background The protein encoded by this gene functions in the second step of the mitochondrial fatty acid beta-oxidation pathway. It catalyzes the hydration of 2-trans-enoyl-coenzyme A (CoA) intermediates to L-3-hydroxyacyl-CoAs. The gene product is a member of the hydratase/isomerase superfamily. It localizes to the mitochondrial matrix. Transcript variants utilizing alternative transcription initiation sites have been described in the literature.
Synonyms ECHS1; enoyl CoA hydratase, short chain, 1, mitochondrial; enoyl Coenzyme A hydratase, short chain, 1, mitochondrial; enoyl-CoA hydratase, mitochondrial; SCEH; C80529; EC; ECHS 1; Enoyl CoA hydratase 1; Enoyl CoA hydratase mitochondrial; Enoyl CoA hydratase short chain 1 mitochondrial; Enoyl Coenzyme A hydratase short chain 1; Enoyl Coenzyme A hydratase short chain 1 mitochondrial; Mitochondrial short chain enoyl coenzyme A hydratase 1; OTTHUMP00000020811; Short chain enoyl CoA hydratase; enoyl-CoA hydratase 1; short-chain enoyl-CoA hydratase
    • Species :
    • Chicken
    • Human
    • Mouse
    • Rat
    • Zebrafish
    • Source :
    • E.coli
    • Mammalian Cell
    • Wheat Germ
    • Tag :
    • GST
    • His
    • N/A
    Species Cat.# Product name Source (Host) Tag Protein Length Price
    Human ECHS1-12268H Recombinant Human ECHS1, His-tagged E.coli His
    Human ECHS1-2811H Recombinant Human ECHS1, His-tagged E.coli His
    Human ECHS1-28225TH Recombinant Human ECHS1, His-tagged E.coli His
    Human ECHS1-3034H Recombinant Human ECHS1 Protein, GST-tagged Wheat Germ GST
    Human ECHS1-528HCL Recombinant Human ECHS1 cell lysate N/A
    Mouse ECHS1-4969M Recombinant Mouse ECHS1 Protein Mammalian Cell His
    Rat ECHS1-2004R Recombinant Rat ECHS1 Protein Mammalian Cell His
    Chicken ECHS1-4803C Recombinant Chicken ECHS1 Mammalian Cell His
    Zebrafish ECHS1-1255Z Recombinant Zebrafish ECHS1 Mammalian Cell His

    ECHS1 involved in several pathways and played different roles in them. We selected most pathways ECHS1 participated on our site, such as content, which may be useful for your reference. Also, other proteins which involved in the same pathway with ECHS1 were listed below. Creative BioMart supplied nearly all the proteins listed, you can search them on our site.

    Pathway Name Pathway Related Protein
    Beta oxidation of butanoyl-CoA to acetyl-CoA ECHS1
    Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA ECHS1
    Beta oxidation of hexanoyl-CoA to butanoyl-CoA ECHS1
    Beta oxidation of lauroyl-CoA to decanoyl-CoA-CoA ECHS1
    Beta oxidation of octanoyl-CoA to hexanoyl-CoA ECHS1
    Biosynthesis of antibiotics BPNT1; ADSL; DAO; GPR19; AGXT; CTH; AMDHD2; NME6; DLD; CS
    Butanoate metabolism ACSM3; GAD1B; BDH1; BDH2; OXCT2; HMGCL; AACS; ACSM2; GAD1; ALDH5A1
    Carbon metabolism ackA; RPE; PGP; FBP1B; ALDOC; SDHDA; PHGDH; AGXTB; FBP1; PGAM1B

    ECHS1 has several biochemical functions, for example, Content. Some of the functions are cooperated with other proteins, some of the functions could acted by ECHS1 itself. We selected most functions ECHS1 had, and list some proteins which have the same functions with ECHS1. You can find most of the proteins on our site.

    Function Related Protein
    enoyl-CoA hydratase activity HADHAA; HADHAB; EHHADH; HADHA; ECHS1; HADHB; AUH
    protein binding HEYL; ABCG2; TCL1A; MIER1; BCL9; PSMC3; A2M; IRF5; PITX1; CXorf41

    ECHS1 has direct interactions with proteins and molecules. Those interactions were detected by several methods such as yeast two hybrid, co-IP, pull-down and so on. We selected proteins and molecules interacted with ECHS1 here. Most of them are supplied by our site. Hope this information will be useful for your research of ECHS1.


    Sakai, C; Yamaguchi, S; et al. ECHS1 Mutations Cause Combined Respiratory Chain Deficiency Resulting in Leigh Syndrome. HUMAN MUTATION 36:232-239(2015).
    Xu, WD; Yang, XY; et al. Up-regulation of fatty acid oxidation in the ligament as a contributing factor of ankylosing spondylitis: A comparative proteomic study. JOURNAL OF PROTEOMICS 113:57-72(2015).

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