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Mouse Anti-MPI Recombinant Antibody (CBFYM-2491) (CBMAB-M2678-FY)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
CBFYM-2491
Antibody Isotype
IgG1
Application
WB, IHC, FC

Basic Information

Immunogen
Full length human recombinant protein of human MPI(NP_002426) produced in HEK293T cell
Specificity
Human
Antibody Isotype
IgG1
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
Buffer
PBS, pH 7.3, 1% BSA
Preservative
0.02% Sodium azide
Concentration
0.73 mg/mL
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Mannose Phosphate Isomerase
Introduction
Phosphomannose isomerase catalyzes the interconversion of fructose-6-phosphate and mannose-6-phosphate and plays a critical role in maintaining the supply of D-mannose derivatives, which are required for most glycosylation reactions. Mutations in the MPI gene were found in patients with carbohydrate-deficient glycoprotein syndrome, type Ib. Alternative splicing results in multiple transcript variants.
Entrez Gene ID
4351
UniProt ID
P34949
Alternative Names
Mannose Phosphate Isomerase; Mannose-6-Phosphate Isomerase; Phosphohexomutase; EC 5.3.1.8; PMI1; PMI
Function
Involved in the synthesis of the GDP-mannose and dolichol-phosphate-mannose required for a number of critical mannosyl transfer reactions.
Biological Process
GDP-mannose biosynthetic process Source: GO_Central
Mannose to fructose-6-phosphate metabolic process Source: Ensembl
Cellular Location
Cytoplasm
Involvement in disease
Congenital disorder of glycosylation 1B (CDG1B):
A form of congenital disorder of glycosylation, a multisystem disorder caused by a defect in glycoprotein biosynthesis and characterized by under-glycosylated serum glycoproteins. Congenital disorders of glycosylation result in a wide variety of clinical features, such as defects in the nervous system development, psychomotor retardation, dysmorphic features, hypotonia, coagulation disorders, and immunodeficiency. The broad spectrum of features reflects the critical role of N-glycoproteins during embryonic development, differentiation, and maintenance of cell functions. CDG1B is clinically characterized by protein-losing enteropathy.
More Infomation

Lu, S., Liang, S., Wu, Y., Liu, J., Lin, L., Huang, G., & Ning, H. (2023). Mannose phosphate isomerase gene mutation leads to a congenital disorder of glycosylation: A rare case report and literature review. Frontiers in Pediatrics, 11, 1150367.

De Graef, D., Mousa, J., Waberski, M. B., & Morava, E. (2022). Mannose treatment improves immune deficiency in mannose phosphate isomerase–congenital disorder of glycosylation: case report and review of literature. Therapeutic Advances in Rare Disease, 3, 26330040221091283.

Luo, H., Wang, X., Wang, Y., Dan, Q., & Ge, H. (2022). Mannose enhances the radio-sensitivity of esophageal squamous cell carcinoma with low MPI expression by suppressing glycolysis. Discover Oncology, 13(1), 1.

Li, Z., Liu, X., Nakanishi, H., & Gao, X. D. (2020). Encapsulation of mannose-6-phosphate isomerase in yeast spores and its application in L-ribose production. Journal of Agricultural and Food Chemistry, 68(25), 6892-6899.

Kim, S. J., Kim, Y. S., & Yeom, S. J. (2020). Phosphate sugar isomerases and their potential for rare sugar bioconversion. Journal of Microbiology, 58, 725-733.

Čechová, A., Altassan, R., Borgel, D., Bruneel, A., Correia, J., Girard, M., ... & Honzík, T. (2020). Consensus guideline for the diagnosis and management of mannose phosphate isomerase‐congenital disorder of glycosylation. Journal of inherited metabolic disease, 43(4), 671-693.

Mühlhausen, C., Henneke, L., Schlotawa, L., Behme, D., Grüneberg, M., Gärtner, J., & Marquardt, T. (2020). Mannose phosphate isomerase deficiency‐congenital disorder of glycosylation (MPI‐CDG) with cerebral venous sinus thrombosis as first and only presenting symptom: A rare but treatable cause of thrombophilia. JIMD reports, 55(1), 38-43.

Noman, K., Hendriksz, C. J., Radcliffe, G., Roncaroli, F., Moreea, S., Hussain, A., & Stepien, K. M. (2020). Clinical outcomes in an adult patient with mannose phosphate isomerase-congenital disorder of glycosylation who discontinued mannose therapy. Molecular Genetics and Metabolism Reports, 25, 100646.

DeRossi, C., Bambino, K., Morrison, J., Sakarin, I., Villacorta‐Martin, C., Zhang, C., ... & Chu, J. (2019). Mannose phosphate isomerase and mannose regulate hepatic stellate cell activation and fibrosis in zebrafish and humans. Hepatology, 70(6), 2107-2122.

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

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