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AB100962

重组人 ECHS1 蛋白 (His tag N-Terminus)

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.

查看别名

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.

关键信息

纯度

>95% SDS-PAGE

ab100962 is purified using conventional chromatography techniques.

表达系统

Escherichia coli

标签

His tag N-Terminus

应用

Mass Spec, SDS-PAGE

applications

生物活性

No

访问

P30084

不含动物源

No

不含载体蛋白

No

种属

Human

存储溶液

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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搭配使用产品

Primary Antibodies

AB99887

Anti-TNS4/CTEN antibody [SP83]

RabMAb|Recombinant

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-TNS4/CTEN antibody [SP83] (AB99887)

  • 5
  • 1
Biochemicals

AB120476

Pro-myristic acid, Negative control for MiTMAB™ and OcTMAB™

Chemical Structure - Pro-myristic acid, Negative control for MiTMAB™ and OcTMAB™ (AB120476)

  • 0
  • 0
Primary Antibodies

AB315396

Anti-Plasma Kallikrein 1B antibody [RM2034]

BOND RX™ Validated|20ul selling size|RabMAb|Recombinant

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Plasma Kallikrein 1B antibody [RM2034] (AB315396)

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  • 0
Primary Antibodies

AB228631

Anti-ECHS1 antibody

Immunocytochemistry/ Immunofluorescence - Anti-ECHS1 antibody (AB228631)

  • 0
  • 0
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反应性数据

{ "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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序列信息

[{"linker":null,"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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性能和储存信息

形式
Liquid
运输条件
Blue Ice
推荐的短期储存条件
-20°C
推荐的长期储存条件
-20°C
分装信息
Upon delivery aliquot
储存信息
Avoid freeze / thaw cycle
False
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补充信息

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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常规信息

功能

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).

序列相似性

Belongs to the enoyl-CoA hydratase/isomerase family.

亚细胞定位

Mitochondrion matrix

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产品实验方案

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靶点信息

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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