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

Name: Mouse Anti-MOCS2 (AA 1-88) Recombinant Antibody (CBFYM-2461)
SKU: CBMAB-M2648-FY
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

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

Basic Information

Host Animal

Mouse

Clone

CBFYM-2461

Application

ELISA, WB

Immunogen

Full length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.Immunogen sequence: MVPLCQVEVL YFAKSAEITG VRSETISVPQ EIKALQLWKE IETRHPGLAD VRNQIIFAVR QEYVELGDQL LVLQPGDEIA VIPPISGG

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 1-88

More Infomation

Target

Full Name

molybdenum cofactor synthesis 2

Introduction

Eukaryotic molybdoenzymes use a unique molybdenum cofactor consisting of a pterin, termed molybdopterin, and the catalytically active metal molybdenum. MoCo is synthesized from precursor Z by the heterodimeric enzyme molybdopterin synthase. The large and small subunits of molybdopterin synthase are both encoded from this gene by overlapping open reading frames. The proteins were initially thought to be encoded from a bicistronic transcript. They are now thought to be encoded from monocistronic transcripts. Alternatively spliced transcripts have been found for this locus that encode the large and small subunits.

Entrez Gene ID

4338

UniProt ID

O96007

Alternative Names

Molybdenum Cofactor Synthesis 2; MCBPE; MOCO1; Molybdenum Cofactor Synthesis Protein 2 Large Subunit; Molybdenum Cofactor Synthesis Protein 2 Small Subunit; Molybdopterin Synthase Sulfur Carrier Subunit; Molybdenum Cofactor Biosynthesis Protein E; Molybdopterin Synthase Catalytic Subunit; Molybdenum Cofactor Synthesis Protein 2A; Molybdenum Cofactor Synthesis Protein 2B; Molybdopterin-Synthase Large Subunit

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)
Proteogenomics
Other Protocols

Isotype control

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

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