Rabbit Polyclonal ECHS1 antibody. Suitable for IHC-P, WB, ICC/IF and reacts with Human, Mouse samples. Cited in 1 publication. Immunogen corresponding to Recombinant Fragment Protein within Human Enoyl-CoA hydratase, mitochondrial aa 50 to C-terminus.
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Publications
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- Journal of medical genetics 52:691-82015Clinical, biochemical and metabolic characterisation of a mild form of human short-chain enoyl-CoA hydratase deficiency: significance of increased N-acetyl-S-(2-carboxypropyl)cysteine excretion.Applications:Unspecified applicationReactive species:Unspecified reactive speciesKenichiro Yamada et. al.PubMed 26251176
Key facts
- Isotype
- IgG
- Host species
- Rabbit
- Storage buffer
pH: 7
Preservative: 0.01% Thimerosal (merthiolate)
Constituents: 20% Glycerol (glycerin, glycerine), 1.21% Tris, 0.75% Glycine- Form
- Liquid
- Clonality
- Polyclonal
Immunogen
- Recombinant Fragment Protein within Human Enoyl-CoA hydratase, mitochondrial aa 50 to C-terminus. The exact immunogen used to generate this antibody is proprietary information. Database link P30084
Reactivity data
Select an application| IHC-P | WB | ICC/IF | |
|---|---|---|---|
| Human | Tested | Tested | Tested |
| Mouse | Expected | Tested | Expected |
IHC-P
TestedTested
| Species | Dilution info | Notes |
|---|---|---|
Species Human | Dilution info 1/100.00000 - 1/1000.00000 | Notes 10mM Citrate buffer Perform heat-mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol. |
ExpectedExpected
| Species | Dilution info | Notes |
|---|---|---|
Species Mouse | Dilution info Use at an assay dependent concentration. | Notes - |
WB
TestedTested
| Species | Dilution info | Notes |
|---|---|---|
Species Mouse | Dilution info 1/500.00000 - 1/3000.00000 | Notes - |
Species Human | Dilution info 1/500.00000 - 1/3000.00000 | Notes - |
ICC/IF
TestedTested
| Species | Dilution info | Notes |
|---|---|---|
Species Human | Dilution info 1/100.00000 - 1/1000.00000 | Notes - |
ExpectedExpected
| Species | Dilution info | Notes |
|---|---|---|
Species Mouse | Dilution info Use at an assay dependent concentration. | Notes - |
Associated Products
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4 products for Alternative Product
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
Recommended products
Rabbit Polyclonal ECHS1 antibody. Suitable for IHC-P, WB, ICC/IF and reacts with Human, Mouse samples. Cited in 1 publication. Immunogen corresponding to Recombinant Fragment Protein within Human Enoyl-CoA hydratase, mitochondrial aa 50 to C-terminus.
Key facts
- Isotype
- IgG
- Host species
- Rabbit
- Storage buffer
pH: 7
Preservative: 0.01% Thimerosal (merthiolate)
Constituents: 20% Glycerol (glycerin, glycerine), 1.21% Tris, 0.75% Glycine- Form
- Liquid
- Clonality
- Polyclonal
- Immunogen
- Recombinant Fragment Protein within Human Enoyl-CoA hydratase, mitochondrial aa 50 to C-terminus. The exact immunogen used to generate this antibody is proprietary information. Database link P30084
- Purification technique
- Affinity purification Immunogen
- Concentration
Storage
- Shipped at conditions
- Blue Ice
- Appropriate short-term storage conditions
- +4°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.
Product promise
Tested
We have tested this species and application combination and it works. It is covered by our product promise.
Expected
We have not tested this specific species and application combination in-house, but expect it will work. It is covered by our product promise.
Predicted
This species and application combination has not been tested, but we predict it will work based on strong homology. However, this combination is not covered by our product promise.
Not recommended
We do not recommend this combination. It is not covered by our product promise.
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4 product images
Immunocytochemistry/ Immunofluorescence - Anti-ECHS1 antibody (ab153732)
Immunofluorescent analysis of methanol-fixed HeLa cells, labeling ECHS1 with ab153732 at 1/200 dilution. Right cells co-stained with Hoechst 33342.
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-ECHS1 antibody (ab153732)
Immunohistochemical analysis of paraffin-embedded Human NCI-N87 xenograft tissue, labeling ECHS1 with ab153732 at 1/250 dilution.
Western blot - Anti-ECHS1 antibody (ab153732)
12% SDS PAGE
All lanes: Western blot - Anti-ECHS1 antibody (ab153732) at 1/1000 dilution
Lane 1: Neuro2A whole cell lysate at 30 µg
Lane 2: GL261 whole cell lysate at 30 µg
Lane 3: C8D30 whole cell lysate at 30 µg
Predicted band size: 31 kDa
Western blot - Anti-ECHS1 antibody (ab153732)
12% SDS PAGE
All lanes: Western blot - Anti-ECHS1 antibody (ab153732) at 1/3000 dilution
Lane 1: NT2D1 whole cell lysate at 30 µg
Lane 2: PC3 whole cell lysate at 30 µg
Lane 3: U-87 MG whole cell lysate at 30 µg
Lane 4: SK N SH whole cell lysate at 30 µg
Predicted band size: 31 kDa
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