Mouse Anti-MVK Recombinant Antibody (2C5) (CBMAB-A5699-LY)

Name: Mouse Anti-MVK Recombinant Antibody (2C5)
SKU: CBMAB-A5699-LY
Rating: 5 (1 reviews)

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Datasheet

SDS

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

Basic Information

Host Animal

Mouse

Clone

2C5

Application

WB, ELISA

Immunogen

MVK (AAH16140, 297 a.a. ~ 396 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Specificity

Human

Antibody Isotype

IgG2a, κ

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

Purity

> 95% Purity determined by SDS-PAGE.

Storage

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

More Infomation

Target

Full Name

Mevalonate Kinase

Introduction

This gene encodes the peroxisomal enzyme mevalonate kinase. Mevalonate is a key intermediate, and mevalonate kinase a key early enzyme, in isoprenoid and sterol synthesis. Mevalonate kinase deficiency caused by mutation of this gene results in mevalonic aciduria, a disease characterized psychomotor retardation, failure to thrive, hepatosplenomegaly, anemia and recurrent febrile crises. Defects in this gene also cause hyperimmunoglobulinaemia D and periodic fever syndrome, a disorder characterized by recurrent episodes of fever associated with lymphadenopathy, arthralgia, gastrointestinal dismay and skin rash. Two transcript variants that encode the same protein have been found for this gene. [provided by RefSeq]

Entrez Gene ID

4598

UniProt ID

Q03426

Alternative Names

FLJ96772; LRBP; MK; MVLK

Function

Catalyzes the phosphorylation of mevalonate to mevalonate 5-phosphate, a key step in isoprenoid and cholesterol biosynthesis (PubMed:9325256, PubMed:18302342, PubMed:9392419, PubMed:11278915).

Biological Process

Cholesterol biosynthetic process Source: UniProtKB
Isopentenyl diphosphate biosynthetic process, mevalonate pathway Source: GO_Central
Isoprenoid biosynthetic process Source: UniProtKB
Negative regulation of inflammatory response Source: BHF-UCL

Cellular Location

Cytoplasm
Peroxisome

Involvement in disease

Mevalonic aciduria (MEVA):
Accumulation of mevalonic acid which causes a variety of symptoms such as psychomotor retardation, dysmorphic features, cataracts, hepatosplenomegaly, lymphadenopathy, anemia, hypotonia, myopathy, and ataxia.
Hyperimmunoglobulinemia D and periodic fever syndrome (HIDS):
Autosomal recessive disease characterized by recurrent episodes of unexplained high fever associated with skin rash, diarrhea, adenopathy (swollen, tender lymph nodes), arthralgias and/or arthritis. Concentration of IgD, and often IgA, are above normal.
Porokeratosis 3, multiple types (POROK3):
A form of porokeratosis, a disorder of faulty keratinization characterized by one or more atrophic patches surrounded by a distinctive hyperkeratotic ridgelike border called the cornoid lamella. The keratotic lesions can progress to overt cutaneous neoplasms, typically squamous cell carcinomas. Multiple clinical variants of porokeratosis are recognized, including porokeratosis of Mibelli, linear porokeratosis, disseminated superficial actinic porokeratosis, palmoplantar porokeratosis, and punctate porokeratosis. Different clinical presentations can be observed among members of the same family. Individuals expressing more than one variant have also been reported.

Touitou, I. (2022). Twists and turns of the genetic story of mevalonate kinase-associated diseases: A review. Genes & Diseases, 9(4), 1000-1007.

Elhani, I., Hentgen, V., Grateau, G., & Georgin-Lavialle, S. (2022). Neurological manifestations in mevalonate kinase deficiency: A systematic review. Molecular Genetics and Metabolism, 136(2), 85-93.

Boursier, G., Rittore, C., Milhavet, F., Cuisset, L., & Touitou, I. (2021). Mevalonate kinase-associated diseases: hunting for phenotype–genotype correlation. Journal of Clinical Medicine, 10(8), 1552.

Politiek, F. A., & Waterham, H. R. (2021). Compromised protein prenylation as pathogenic mechanism in mevalonate kinase deficiency. Frontiers in immunology, 3504.

Tabatabaee, S., Iranbakhsh, A., Shamili, M., & Ardebili, Z. O. (2021). Copper nanoparticles mediated physiological changes and transcriptional variations in microRNA159 (miR159) and mevalonate kinase (MVK) in pepper; potential benefits and phytotoxicity assessment. Journal of Environmental Chemical Engineering, 9(5), 106151.

Govindaraj, G. M., Jain, A., Peethambaran, G., Bhoyar, R. C., Vellarikkal, S. K., Ganapati, A., ... & Scaria, V. (2020). Spectrum of clinical features and genetic variants in mevalonate kinase (MVK) gene of South Indian families suffering from Hyperimmunoglobulin D Syndrome. PloS one, 15(8), e0237999.

Munoz, M. A., Jurczyluk, J., Simon, A., Hissaria, P., Arts, R. J., Coman, D., ... & Rogers, M. J. (2019). Defective protein prenylation in a spectrum of patients with mevalonate kinase deficiency. Frontiers in immunology, 10, 1900.

McClory, J., Lin, J. T., Timson, D. J., Zhang, J., & Huang, M. (2019). Catalytic mechanism of mevalonate kinase revisited, a QM/MM study. Organic & Biomolecular Chemistry, 17(9), 2423-2431.

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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)
Proteogenomics
Other Protocols

Antibody Pairs

MVK Matched Antibody Pair (766) (CAT#: APMAB-766LY)

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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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