Human MOCS2 ELISA Kit (V2LY-0626-LY4901)

Name: Human MOCS2 ELISA Kit
SKU: V2LY-0626-LY4901
Rating: 5 (1 reviews)

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Tested Data

Datasheet

SDS

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

Basic Information

Sensitivity

0.0092 ng/mL

Detection Range

0.02-3.5 ng/mL

Sample Type

Serum, Plasma, cell culture supernates

Specificity

Human

Assay Type

Sandwich

Reactivity

Human

Assay Time

1.5 h

Molecule Mass

20.9 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

molybdenum cofactor synthesis 2

Function

Acts as a sulfur carrier required for molybdopterin biosynthesis. Component of the molybdopterin synthase complex that catalyzes the conversion of precursor Z into molybdopterin by mediating the incorporation of 2 sulfur atoms into precursor Z to generate a dithiolene group. In the complex, serves as sulfur donor by being thiocarboxylated (-COSH) at its C-terminus by MOCS3. After interaction with MOCS2B, the sulfur is then transferred to precursor Z to form molybdopterin.

Biological Process

Mo-molybdopterin cofactor biosynthetic process Source: UniProtKB

Cellular Location

Cytosol

Involvement in disease

Molybdenum cofactor deficiency, complementation group B (MOCODB):
An autosomal recessive metabolic disorder characterized by neonatal onset of intractable seizures, opisthotonus, and facial dysmorphism associated with hypouricemia and elevated urinary sulfite levels. Affected individuals show severe neurologic damage and often die in early childhood.

PTM

C-terminal thiocarboxylation occurs in 2 steps, it is first acyl-adenylated (-COAMP) via the hesA/moeB/thiF part of MOCS3, then thiocarboxylated (-COSH) via the rhodanese domain of MOCS3.

Tofilo, M., Voronova, N., Nigmatullina, L., Kuznetsova, E., Timonina, V., Efimenko, B., ... & Mazunin, I. (2023). Live Birth of a Healthy Child in a Couple with Identical mtDNA Carrying a Pathogenic c. 471_477delTTTAAAAinsG Variant in the MOCS2 Gene. Genes, 14(3), 720.

Suganuma, T. (2022). Beyond Moco Biosynthesis―Moonlighting Roles of MoaE and MOCS2. Molecules, 27(12), 3733.

Suganuma, T., Swanson, S. K., Gogol, M., Garrett, T. J., Florens, L., & Workman, J. L. (2021). MOCS2 links nucleotide metabolism to nucleoli function. Journal of Molecular Cell Biology, 13(11), 838-840.

Lee, H. F., Hsu, C. C., Chi, C. S., & Tsai, C. R. (2021). Genotype-Phenotype Dissociation in Two Taiwanese Children with Molybdenum Cofactor Deficiency Caused by MOCS2 Mutation. Neuropediatrics, 53(03), 200-203.

Jezela-Stanek, A., Blaz, W., Gora, A., Bochenska, M., Kusmierska, K., & Sykut-Cegielska, J. (2020). Proteins Structure Models in the Evaluation of Novel Variant (C. 472_477del) in the MOCS2 Gene. Diagnostics, 10(10), 821.

Jezela-Stanek, A., Blaz, W., Gora, A., Bochenska, M., Kusmierska, K., & Sykut-Cegielska, J. (2020). Novel variant (c. 472_477del) in the MOCS2 gene. Authorea Preprints.

Arican, P., Gencpinar, P., Kirbiyik, O., Yilmaz, S. B., Ersen, A., Oztekin, O., & Dundar, N. O. (2019). The clinical and molecular characteristics of molybdenum cofactor deficiency due to MOCS2 mutations. Pediatric neurology, 99, 55-59.

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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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Mouse Anti-MOCS2 (AA 1-88) Recombinant Antibody (CBFYM-2461) (CAT#: CBMAB-M2648-FY)

Isotype control

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

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