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AB100962

Recombinant Human ECHS1 protein (His tag N-Terminus)

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Recombinant Human ECHS1 protein (His tag N-Terminus) is a Human Full Length protein, in the 28 to 290 aa range, expressed in Escherichia coli, with >95%, suitable for SDS-PAGE, Mass Spec.

View Alternative Names

mECH, mECH1, Enoyl-CoA hydratase 1, Short-chain enoyl-CoA hydratase, ECHS1, SCEH

1 Images
SDS-PAGE - Recombinant Human ECHS1 protein (His tag N-Terminus) (AB100962)
  • SDS-PAGE

Unknown

SDS-PAGE - Recombinant Human ECHS1 protein (His tag N-Terminus) (AB100962)

15% SDS-PAGE showing ab100962 (3 μg) at approximately 30.6 kDa.

Key facts

Purity

>95% SDS-PAGE

Expression system

Escherichia coli

Tags

His tag N-Terminus

Applications

SDS-PAGE, Mass Spec

applications

Biologically active

No

Accession

P30084

Animal free

No

Carrier free

No

Species

Human

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

storage-buffer

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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Reactivity", "Dilution Info", "Notes"] }, "values": { "SDS-PAGE": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" }, "Mass Spec": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" } } }
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Sequence info

[{"sequence":"MGSSHHHHHHSSGLVPRGSHMASGANFEYIIAEKRGKNNTVGLIQLNRPKALNALCDGLIDELNQALKIFEEDPAVGAIVLTGGDKAFAAGADIKEMQNLSFQDCYSSKFLKHWDHLTQVKKPVIAAVNGYAFGGGCELAMMCDIIYAGEKAQFAQPEILIGTIPGAGGTQRLTRAVGKSLAMEMVLTGDRISAQDAKQAGLVSKICPVETLVEEAIQCAEKIASNSKIVVAMAKESVNAAFEMTLTEGSKLEKKLFYSTFATDDRKEGMTAFVEKRKANFKDQ","proteinLength":"Full Length","predictedMolecularWeight":"30.6 kDa","actualMolecularWeight":null,"aminoAcidEnd":290,"aminoAcidStart":28,"nature":"Recombinant","expressionSystem":"Escherichia coli","accessionNumber":"P30084","tags":[{"tag":"His","terminus":"N-Terminus"}]}]
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Properties and storage information

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
False
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Supplementary information

This supplementary information is collated from multiple sources and compiled automatically.

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.

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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Specifications

Form

Liquid

Additional notes

ab100962 is purified using conventional chromatography techniques.

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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

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Product protocols

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Target data

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).
See full target information Enoyl-CoA hydratase, mitochondrial
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Complementary Products

Primary Antibodies

AB174312

Anti-ECHS1 antibody [EPR11785]

RabMAb|Recombinant

Immunocytochemistry/ Immunofluorescence - Anti-ECHS1 antibody [EPR11785] (AB174312)

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Proteins & Peptides

AB98118

Recombinant Human GCDH/GCD protein (His tag N-Terminus)

SDS-PAGE - Recombinant Human GCDH/GCD protein (AB98118)

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Secondary Antibodies

AB150077

Goat Anti-Rabbit IgG H&L (Alexa Fluor® 488)

Immunocytochemistry/ Immunofluorescence - Goat Anti-Rabbit IgG H&L (Alexa Fluor® 488) (AB150077)

  • 5
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Secondary Antibodies

AB150121

Goat Anti-Mouse IgM mu chain (Alexa Fluor® 488)

Immunocytochemistry/ Immunofluorescence - Goat Anti-Mouse IgM mu chain (Alexa Fluor® 488) (AB150121)

  • 5
  • 5

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