Anti-MCCC2 Antibody

$99.00$319.00

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

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Rabbit Anti-MCCC2 Antibody (CSB-PA867199ESR1HU)

Supplier: CUSABIO BIOTECH CO.

Alternative names:

Methylcrotonoyl-CoA carboxylase beta chain Antibody,mitochondrial Antibody,MCCase subunit beta Antibody,3-methylcrotonyl-CoA carboxylase 2 Antibody,3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit Antibody,3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta Antibody,MCCC2 Antibody,MCCB Antibody

More alternative names for the antibody
3 methylcrotonyl CoA carboxylase 2 antibody|3 methylcrotonyl CoA carboxylase non biotin containing subunit antibody|3 methylcrotonyl CoA:carbon dioxide ligase subunit beta antibody|3-methylcrotonyl-CoA carboxylase 2 antibody|3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit antibody|3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta antibody|Biotin carboxylase antibody|MCCase subunit beta antibody|MCCB antibody|MCCB_HUMAN antibody|MCCC 2 antibody|Mccc2 antibody|Methylcrotonoyl CoA carboxylase 2 (beta) antibody|Methylcrotonoyl CoA carboxylase beta chain mitochondrial antibody|Methylcrotonoyl Coenzyme A carboxylase 2 (beta) antibody|Methylcrotonoyl-CoA carboxylase beta chain antibody|mitochondrial antibody|Non biotin containing subunit of 3 methylcrotonyl CoA carboxylase antibody
Anti-MCCC2 antibody (ab197297)
Close sc-390836|sc-50738|sc-366942|

Recommended applications: ELISA, WB, IHC

Recommended dilution: User optimized

Recommended protocols: check protocols

Name

Anti-MCCC2 Antibody

Catalogue No.

CSB-PA867199ESR1HU

Reactivity

Human, Mouse

Immunogen

Recombinant human Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial protein (284-563AA)

Host

Rabbit

Applications

ELISA, WB, IHC

Recommended dilution

User optimized

Clonality

Polyclonal

Conjugation

unconjugated

Isotype

IgG

Molecular weight

61 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:64087( Human), Entrez Gene:78038( Mouse), Entrez Gene:361884( Rat), Omim:609014( Human), SwissProt:Q9HCC0( Human), SwissProt:Q3ULD5( Mouse), SwissProt:Q5XIT9( Rat), Unigene:604789( Human), Unigene:137327( Mouse), Unigene:33635( Rat)

Protein function

Carboxyltransferase subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism. .

Involvement in disease

3-methylcrotonoyl-CoA carboxylase 2 deficiency (MCC2D) [MIM:210210]: An autosomal recessive disorder of leucine catabolism. The phenotype is variable, ranging from neonatal onset with severe neurological involvement to asymptomatic adults. There is a characteristic organic aciduria with massive excretion of 3-hydroxyisovaleric acid and 3-methylcrotonylglycine, usually in combination with a severe secondary carnitine deficiency. . Note=The disease is caused by mutations affecting the gene represented in this entry.

Protein sequence and domain

Belongs to the AccD/PCCB family.

Protein cellular localization

Mitochondrion matrix .

Research area

All research areas>Transcription Regulators>MCCB
(View all antibody categories related to Transcription Regulators)

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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