echs1
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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 4.2.1.17; 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
Cat.#:ECHS1-3034HTag:GST
Source (Host):Wheat GermSpecies:Human
Product nameRecombinant Human ECHS1 Protein, GST-tagged
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Cat.#:ECHS1-2811HTag:His
Source (Host):E. coliSpecies:Human
Product nameRecombinant Human ECHS1, His-tagged
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Cat.#:ECHS1-12268HTag:His
Source (Host):E. coliSpecies:Human
Product nameRecombinant Human ECHS1, His-tagged
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Cat.#:ECHS1-28225THTag:His
Source (Host):E. coliSpecies:Human
Product nameRecombinant Human ECHS1, His-tagged
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Cat.#:ECHS1-4969MTag:His
Source (Host):Mammalian CellsSpecies:Mouse
Product nameRecombinant Mouse ECHS1 Protein
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Cat.#:ECHS1-2004RTag:His
Source (Host):Mammalian CellsSpecies:Rat
Product nameRecombinant Rat ECHS1 Protein
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Cat.#:ECHS1-4803CTag:His
Source (Host):Mammalian CellsSpecies:Chicken
Product nameRecombinant Chicken ECHS1
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Cat.#:ECHS1-1255ZTag:His
Source (Host):Mammalian CellsSpecies:Zebrafish
Product nameRecombinant Zebrafish ECHS1
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Cat.#:ECHS1-528HCLTag:
Source (Host):Species:Human
Product nameRecombinant Human ECHS1 cell lysate
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Involved Pathway

ECHS1 involved in several pathways and played different roles in them. We selected most pathways ECHS1 participated on our site, such as Beta oxidation of butanoyl-CoA to acetyl-CoA, Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA, Beta oxidation of hexanoyl-CoA to butanoyl-CoA, 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
GLYCTK; CTH; DLD; GPR19; AKR1A1; AMDHD2; SDSL; AK7; DLAT; DBT
Butanoate metabolism
HADHA; ACADS; HADH; OXCT1A; Acsm2a; ACSM3; ALDH5A1; BDH1; HMGCLL1; ACSM2B
Carbon metabolism
FBP1A; GLUD1; SDS; HKDC1; PGAM1; ENO1B; ESD; PRPS1A; ACSS2; AGXT

Protein Function

ECHS1 has several biochemical functions, for example, enoyl-CoA hydratase activity, protein binding. 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
Function enoyl-CoA hydratase activity
Related Protein ECHS1; HADHA; HADHB; HADHAA; EHHADH; AUH; HADHAB
Function protein binding
Related Protein TIA1; PLOD3; FYCO1; HIPK1; MMRN2; STXBP3; DERL3; STX19; PPP4C; ZBTB25

Interacting Protein

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.
LRRK2; Stat3; STAT3; LRRK1; RIPK2; EIF1B; GH1; EPB41; TIMP2; TFE3; MCC; HLA-B; VHL; GABARAPL2; YWHAZ

ECHS1 Related Articles

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).