Anti-ECHS1 Antibody

$99.00$319.00

Reactivity: Human,Mouse,Rat
Applications: ELISA,WB,IHC
Conjugation: Various
Supplier: CUSABIO BIOTECH CO.

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Rabbit Anti-ECHS1 Antibody (CSB-PA007382ESR2HU)

Supplier: CUSABIO BIOTECH CO.

Alternative names:

Enoyl-CoA hydratase Antibody,mitochondrial Antibody,Enoyl-CoA hydratase 1 Antibody,Short-chain enoyl-CoA hydratase Antibody,SCEH Antibody,ECHS1 Antibody

More alternative names for the antibody
C80529 antibody|EC 4.2.1.17 antibody|ECHS 1 antibody|Enoyl CoA hydratase 1 antibody|Enoyl CoA hydratase mitochondrial antibody|Enoyl CoA hydratase short chain 1 mitochondrial antibody|Enoyl Coenzyme A hydratase short chain 1 antibody|Enoyl Coenzyme A hydratase short chain 1 mitochondrial antibody|Mitochondrial short chain enoyl coenzyme A hydratase 1 antibody|OTTHUMP00000020811 antibody|SCEH antibody|Short chain enoyl CoA hydratase antibody
Anti-ECHS1 antibody [EPR11784(B)] (ab170108)
Close sc-133534|

Recommended applications: ELISA, WB, IHC

Recommended dilution: User optimized

Recommended protocols: check protocols

Name

Anti-ECHS1 Antibody

Catalogue No.

CSB-PA007382ESR2HU

Reactivity

Human, Mouse, Rat

Immunogen

Recombinant human Enoyl-CoA hydratase, mitochondrial protein (28-290AA)

Host

Rabbit

Applications

ELISA, WB, IHC

Recommended dilution

User optimized

Clonality

Polyclonal

Conjugation

unconjugated

Isotype

IgG

Molecular weight

31 kDa

Purification

Antigen Affinity Purified

Storage

Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.

Database links

Entrez Gene:1892( Human), Entrez Gene:93747( Mouse), Entrez Gene:140547( Rat), Omim:602292( Human), SwissProt:P30084( Human), SwissProt:Q8BH95( Mouse), SwissProt:P14604( Rat), Unigene:76394( Human), Unigene:24452( Mouse), Unigene:6847( Rat)

Protein function

Straight-chain enoyl-CoA thioesters from C4 up to at least C16 are processed, although with decreasing catalytic rate. Has high substrate specificity for crotonyl-CoA and moderate specificity for acryloyl-CoA, 3-methylcrotonyl-CoA and methacrylyl-CoA. It is noteworthy that binds tiglyl-CoA, but hydrates only a small amount of this substrate. .

Protein tissue specificity

Liver, fibroblast, muscle. Barely detectable in spleen and kidney.

Involvement in disease

Mitochondrial short-chain enoyl-CoA hydratase 1 deficiency (ECHS1D) [MIM:616277]: A severe, autosomal recessive inborn error affecting valine metabolism. Disease features include brain lesions in the basal ganglia, neurodegeneration, delayed psychomotor development, hypotonia, spasticity, and increased lactic acid in serum and cerebral serum fluid. . Note=The disease is caused by mutations affecting the gene represented in this entry.

Protein sequence and domain

Belongs to the enoyl-CoA hydratase/isomerase family.

Protein cellular localization

Mitochondrion matrix.

Research area

All research areas>Synthesis and Degradation>ECH
(View all antibody categories related to Synthesis and Degradation)

Note

AntibodyPlus provides antibody trial sample for your own antibody validation and collects antibody reviews.

Supplier

CUSABIO BIOTECH CO.

Product type

Primary antibody

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