Recombinant Human ECHS1 protein is a Human Full Length protein, in the 28 to 290 aa range, expressed in Escherichia coli, with >95% purity and suitable for SDS-PAGE, MS.
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Key facts
- Purity
- >95% SDS-PAGE
- Expression system
- Escherichia coli
- Tags
- His tag N-Terminus
- Applications
- SDS-PAGE, MS
- Biologically active
- No
Amino acid sequence
M G S S H H H H H H S S G L V P R G S H M A S G A N F E Y I I A E K R G K N N T V G L I Q L N R P K A L N A L C D G L I D E L N Q A L K I F E E D P A V G A I V L T G G D K A F A A G A D I K E M Q N L S F Q D C Y S S K F L K H W D H L T Q V K K P V I A A V N G Y A F G G G C E L A M M C D I I Y A G E K A Q F A Q P E I L I G T I P G A G G T Q R L T R A V G K S L A M E M V L T G D R I S A Q D A K Q A G L V S K I C P V E T L V E E A I Q C A E K I A S N S K I V V A M A K E S V N A A F E M T L T E G S K L E K K L F Y S T F A T D D R K E G M T A F V E K R K A N F K D Q
Reactivity data
| Application | Reactivity | Dilution info | Notes |
|---|---|---|---|
Application SDS-PAGE | Reactivity Reacts | Dilution info - | Notes - |
Application MS | Reactivity Reacts | Dilution info - | Notes - |
Target data
Function
Converts unsaturated trans-2-enoyl-CoA species ((2E)-enoyl-CoA) to the corresponding (3S)-3hydroxyacyl-CoA species through addition of a water molecule to the double bond (PubMed:25125611, PubMed:26251176). Catalyzes the hydration of medium- and short-chained fatty enoyl-CoA thioesters from 4 carbons long (C4) up to C16 (PubMed:26251176). Has high substrate specificity for crotonyl-CoA ((2E)-butenoyl-CoA) and moderate specificity for acryloyl-CoA, 3-methylcrotonyl-CoA (3-methyl-(2E)-butenoyl-CoA) and methacrylyl-CoA ((2E)-2-methylpropenoyl-CoA) (PubMed:26251176). Can bind tiglyl-CoA (2-methylcrotonoyl-CoA), but hydrates only a small amount of this substrate (PubMed:26251176). Plays a key role in the beta-oxidation spiral of short- and medium-chain fatty acid oxidation (PubMed:25125611, PubMed:26251176). At a lower rate than the hydratase reaction, catalyzes the isomerase reaction of trans-3-enoyl-CoA species (such as (3E)-hexenoyl-CoA) to trans-2-enoyl-CoA species (such as (2E)-hexenoyl-CoA), which are subsequently hydrated to 3(S)-3-hydroxyacyl-CoA species (such as (3S)-hydroxyhexanoyl-CoA) (By similarity).
Alternative names
mECH, mECH1, Enoyl-CoA hydratase 1, Short-chain enoyl-CoA hydratase, ECHS1, SCEH
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Recombinant Human ECHS1 protein is a Human Full Length protein, in the 28 to 290 aa range, expressed in Escherichia coli, with >95% purity and suitable for SDS-PAGE, MS.
Key facts
- Purity
- >95% SDS-PAGE
- Expression system
- Escherichia coli
- Tags
- His tag N-Terminus
- Applications
- SDS-PAGE, MS
- Biologically active
- No
- Accession
- P30084-1
- Animal free
- No
- Species
- Human
- Concentration
- Storage buffer
pH: 8
Constituents: 20% Glycerol (glycerin, glycerine), 0.58% Sodium chloride, 0.316% Tris HCl, 0.0154% (R*,R*)-1,4-Dimercaptobutan-2,3-diol
Sequence info
Amino acid sequence
- M G S S H H H H H H S S G L V P R G S H M A S G A N F E Y I I A E K R G K N N T V G L I Q L N R P K A L N A L C D G L I D E L N Q A L K I F E E D P A V G A I V L T G G D K A F A A G A D I K E M Q N L S F Q D C Y S S K F L K H W D H L T Q V K K P V I A A V N G Y A F G G G C E L A M M C D I I Y A G E K A Q F A Q P E I L I G T I P G A G G T Q R L T R A V G K S L A M E M V L T G D R I S A Q D A K Q A G L V S K I C P V E T L V E E A I Q C A E K I A S N S K I V V A M A K E S V N A A F E M T L T E G S K L E K K L F Y S T F A T D D R K E G M T A F V E K R K A N F K D Q
- Accession
- P30084
- Protein length
- Full Length
- Predicted molecular weight
- 30.6 kDa
- Amino acids
- 28 to 290
- Nature
- Recombinant
- Tags
- His tag N-Terminus
Specifications
- Form
- Liquid
- Additional notes
ab100962 is purified using conventional chromatography techniques.
General info
- Function
Converts unsaturated trans-2-enoyl-CoA species ((2E)-enoyl-CoA) to the corresponding (3S)-3hydroxyacyl-CoA species through addition of a water molecule to the double bond (PubMed:25125611, PubMed:26251176). Catalyzes the hydration of medium- and short-chained fatty enoyl-CoA thioesters from 4 carbons long (C4) up to C16 (PubMed:26251176). Has high substrate specificity for crotonyl-CoA ((2E)-butenoyl-CoA) and moderate specificity for acryloyl-CoA, 3-methylcrotonyl-CoA (3-methyl-(2E)-butenoyl-CoA) and methacrylyl-CoA ((2E)-2-methylpropenoyl-CoA) (PubMed:26251176). Can bind tiglyl-CoA (2-methylcrotonoyl-CoA), but hydrates only a small amount of this substrate (PubMed:26251176). Plays a key role in the beta-oxidation spiral of short- and medium-chain fatty acid oxidation (PubMed:25125611, PubMed:26251176). At a lower rate than the hydratase reaction, catalyzes the isomerase reaction of trans-3-enoyl-CoA species (such as (3E)-hexenoyl-CoA) to trans-2-enoyl-CoA species (such as (2E)-hexenoyl-CoA), which are subsequently hydrated to 3(S)-3-hydroxyacyl-CoA species (such as (3S)-hydroxyhexanoyl-CoA) (By similarity).
- Sequence similarities
Belongs to the enoyl-CoA hydratase/isomerase family.
- Subcellular localisation
- Mitochondrion matrix
Storage
- Shipped at conditions
- Blue Ice
- Appropriate short-term storage conditions
- -20°C
- Appropriate long-term storage conditions
- -20°C
- Aliquoting information
- Upon delivery aliquot
- Storage information
- Avoid freeze / thaw cycle
Supplementary info
- This supplementary information is collated from multiple sources and compiled automatically.
- Activity summary
ECHS1 Enoyl-CoA Hydratase mitochondrial also known as short-chain enoyl-CoA hydratase 1 is a mitochondrial enzyme involved in fatty acid metabolism. It has a molecular mass of approximately 30 kDa. This protein is expressed predominantly in tissues with high energy demands like heart and skeletal muscle. ECHS1 catalyzes the hydration of enoyl-CoA to 3-hydroxyacyl-CoA playing a significant role in the beta-oxidation pathway of fatty acid breakdown.
- Biological function summary
ECHS1 functions as an important enzyme in the mitochondrial fatty acid degradation pathway. It participates as a component of the fatty acid beta-oxidation complex where it collaborates with other enzymes to facilitate energy production through lipids. ECHS1 ensures the conversion of unsaturated fatty acids into metabolites usable in subsequent steps of the energy cycle supporting cellular metabolism.
- Pathways
ECHS1 lies within the beta-oxidation pathway of fatty acids important for the energy supply in mitochondria. This pathway directly links to the citric acid cycle (Krebs cycle) where acetyl-CoA produced through beta-oxidation gets further oxidized for energy generation. ECHS1 functions in concert with other enzymes such as Acyl-CoA dehydrogenases that precede its action in the pathway ensuring complete fatty acid degradation.
- Associated diseases and disorders
Defects in ECHS1 activity associate with rare metabolic disorders like short-chain enoyl-CoA hydratase deficiency. This deficiency leads to a build-up of toxic metabolites affecting the nervous system and muscle function. Moreover abnormalities in ECHS1 expression can relate to mitochondrial diseases impacting proteins such as Pyruvate dehydrogenase which further disrupts energy metabolism. Understanding ECHS1's role in these processes has important implications for diagnosing and developing treatments for metabolic conditions.
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1 product image
SDS-PAGE - Recombinant Human ECHS1 protein (ab100962)
15% SDS-PAGE showing ab100962 (3 μg) at approximately 30.6 kDa.
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