Mouse Anti-MCCC2 (AA 456-564) Recombinant Antibody (CBFYM-1865) (CBMAB-M2036-FY)

Name: Mouse Anti-MCCC2 (AA 456-564) Recombinant Antibody (CBFYM-1865)
SKU: CBMAB-M2036-FY
Rating: 5 (1 reviews)

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Datasheet

Summary

Host Animal

Mouse

Specificity

Human

Clone

CBFYM-1865

Antibody Isotype

IgG1, k

Application

ELISA, IHC-P, WB

Basic Information

Immunogen

Recombinant protein with GST tag

Specificity

Human

Antibody Isotype

IgG1, k

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format

Liquid

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Epitope

AA 456-564

Target

Full Name

methylcrotonoyl-Coenzyme A carboxylase 2 (beta)

Introduction

This gene encodes the small subunit of 3-methylcrotonyl-CoA carboxylase. This enzyme functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. Mutations in this gene are associated with 3-Methylcrotonylglycinuria, an autosomal recessive disorder of leucine catabolism.

Entrez Gene ID

64087

UniProt ID

Q9HCC0

Alternative Names

Methylcrotonoyl-CoA Carboxylase 2; 3-Methylcrotonyl-CoA Carboxylase Non-Biotin-Containing Subunit; 3-Methylcrotonyl-CoA:Carbon Dioxide Ligase Subunit Beta; Methylcrotonoyl-Coenzyme A Carboxylase 2 (Beta); 3-Methylcrotonyl-CoA Carboxylase 2; MCCase Subunit Beta; EC 6.4.1.4; MCCB

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.

Biological Process

Branched-chain amino acid catabolic process Source: ComplexPortal
Coenzyme A metabolic process Source: Ensembl
Leucine catabolic process Source: GO_Central

Cellular Location

Mitochondrion matrix

Involvement in disease

3-methylcrotonoyl-CoA carboxylase 2 deficiency (MCC2D):
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.

More Infomation

References

Liu, W., Chen, S., Xie, W., Wang, Q., Luo, Q., Huang, M., ... & Chen, D. (2023). MCCC2 is a novel mediator between mitochondria and telomere and functions as an oncogene in colorectal cancer. Cellular & Molecular Biology Letters, 28(1), 80.

Chen, Y. Y., Zhang, X. N., Xu, C. Z., Zhou, D. H., Chen, J., Liu, Z. X., ... & Qu, L. S. (2021). MCCC2 promotes HCC development by supporting leucine oncogenic function. Cancer cell international, 21, 1-13.

Li, R., Xu, Z., Zhao, D., Zhang, Y., Xie, Z., Wang, C., ... & Song, J. (2021). Analysis of MCCC2 gene variant in a pedigree affected with 3-methylcrotonyl coenzyme A carboxylase deficiency. Zhonghua yi xue yi Chuan xue za zhi= Zhonghua Yixue Yichuanxue Zazhi= Chinese Journal of Medical Genetics, 38(1), 74-77.

Dai, W., Feng, H., & Lee, D. (2020). MCCC2 overexpression predicts poorer prognosis and promotes cell proliferation in colorectal cancer. Experimental and Molecular Pathology, 115, 104428.

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Protocols

Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

Associated Products

Related Products

Mouse Anti-MCCC2 Recombinant Antibody (2B3)

Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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Documents

Datasheet
SDS
Request for COA

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Second antibody and isotype control

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