Human MCCC1 ELISA Kit (V2LY-0626-LY4886)

Name: Human MCCC1 ELISA Kit
SKU: V2LY-0626-LY4886
Rating: 5 (1 reviews)

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Tested Data

Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Sensitivity

0.0074 ng/mL

Detection Range

0.015-3 ng/mL

Sample Type

Serum, Plasma, cell culture supernates

Specificity

Human

Assay Type

Sandwich

Reactivity

Human

Assay Time

1.5 h

Molecule Mass

80.5 kDa

Components

Pre-coated ELISA plate: 12 wells * 8 detachable strips
Standard solution: 0.5ml x1
Standard diluent: 3ml x1
Streptavidin-HRP: 6ml x1
Stop solution: 6ml x1
Substrate solution A: 6ml x1
Substrate solution B: 6ml x1
Wash buffer concentrate (25x): 20ml x1
Biotinylated antibody: 1ml x1

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

Storage

Store at 2-8°C

More Infomation

Target

Full Name

METHYLCROTONOYL-COA CARBOXYLASE 1

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.

Biological Process

Biotin metabolic process Source: UniProtKB
Branched-chain amino acid catabolic process Source: ComplexPortal
Leucine catabolic process Source: ParkinsonsUK-UCL

Cellular Location

Mitochondrion matrix

Involvement in disease

3-methylcrotonoyl-CoA carboxylase 1 deficiency (MCC1D):
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.

PTM

Acetylated.

Lim, J. L., Ng, E. Y., Lim, S. Y., Tan, A. H., Abdul-Aziz, Z., Ibrahim, K. A., ... & Ahmad-Annuar, A. (2021). Association study of MCCC1/LAMP3 and DGKQ variants with Parkinson’s disease in patients of Malay ancestry. Neurological Sciences, 42, 4203-4207.

Liao, F., Tan, Y., Wang, Y., Zhou, C., Wang, Q., Li, J., ... & Peng, X. (2021). lncRNA AABR07005593. 1 potentiates PM2. 5-induced interleukin-6 expression by targeting MCCC1. Ecotoxicology and Environmental Safety, 226, 112834.

Shao, Z., Masuho, I., Tumber, A., Maynes, J. T., Tavares, E., Ali, A., ... & Vincent, A. (2021). Extended Phenotyping and Functional Validation Facilitate Diagnosis of a Complex Patient Harboring Genetic Variants in MCCC1 and GNB5 Causing Overlapping Phenotypes. Genes, 12(9), 1352.

Zhao, A., Li, Y., Niu, M., Li, G., Luo, N., Zhou, L., ... & Liu, J. (2020). SNPs in SNCA, MCCC1, DLG2, GBF1 and MBNL2 are associated with Parkinson's disease in southern Chinese population. Journal of Cellular and Molecular Medicine, 24(15), 8744-8752.

Chang, K. H., Chen, C. M., Chen, Y. C., Fung, H. C., & Wu, Y. R. (2019). Polymorphisms of ACMSD-TMEM163, MCCC1, and BCKDK-STX1B are not associated with Parkinson’s disease in Taiwan. Parkinson’s Disease, 2019.

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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)
Proteogenomic
Other Protocols

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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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