Anti-MCCC1 Antibody

$99.00$319.00

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

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Rabbit Anti-MCCC1 Antibody (CSB-PA853497ESR1HU)

Supplier: CUSABIO BIOTECH CO.

Alternative names:

Methylcrotonoyl-CoA carboxylase subunit alpha Antibody,mitochondrial Antibody,MCCase subunit alpha Antibody,3-methylcrotonyl-CoA carboxylase 1 Antibody,3-methylcrotonyl-CoA carboxylase biotin-containing subunit Antibody,3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha Antibody,MCCC1 Antibody,MCCA Antibody

More alternative names for the antibody
3-methylcrotonyl-CoA carboxylase 1 antibody|3-methylcrotonyl-CoA carboxylase biotin-containing subunit antibody|3-methylcrotonyl-CoA carboxylase, alpha antibody|3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha antibody|DKFZp686B20267 antibody|FLJ25545 antibody|MCC-B antibody|MCCA antibody|MCCase subunit alpha antibody|methylcrotonoyl-CoA carboxylase 1 (alpha) antibody|methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial antibody|methylcrotonoyl-Coenzyme A carboxylase 1 (alpha) antibody
Anti-MCCC1 antibody (ab178675)
Close sc-365754|sc-46161|sc-376647|sc-66960|sc-46163|sc-46164|

Recommended applications: ELISA, IHC

Recommended dilution: Recommended dilution:IHC:1:20-1:200

Recommended protocols: check protocols

Name

Anti-MCCC1 Antibody

Catalogue No.

CSB-PA853497ESR1HU

Reactivity

Human

Immunogen

Recombinant human Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial protein (526-725AA)

Host

Rabbit

Applications

ELISA, IHC

Recommended dilution

Recommended dilution:IHC:1:20-1:200

Clonality

Polyclonal

Conjugation

unconjugated

Isotype

IgG

Molecular weight

80 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:56922( Human), Entrez Gene:72039( Mouse), Entrez Gene:294972( Rat), Omim:609010( Human), SwissProt:Q96RQ3( Human), SwissProt:Q99MR8( Mouse), SwissProt:Q5I0C3( Rat), Unigene:47649( Human), Unigene:249016( Mouse), Unigene:15587( Rat)

Protein function

Biotin-attachment 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 1 deficiency (MCC1D) [MIM:210200]: 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 cellular localization

Mitochondrion matrix .

Research area

All research areas>Transport and Trafficking>MCCA
(View all antibody categories related to Transport and Trafficking)

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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