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Mouse Anti-MAN1B1 Recombinant Antibody (6B1) (CBMAB-A5183-LY)

The product is antibody recognizes MAN1B1. The antibody 6B1 immunoassay techniques such as: WB, ELISA.
See all MAN1B1 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
6B1
Antibody Isotype
IgG2a, κ
Application
WB, ELISA

Basic Information

Immunogen
MAN1B1 (NP_057303, 112 a.a. ~ 211 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.

Target

Full Name
mannosidase, alpha, class 1B, member 1
Entrez Gene ID
11253
UniProt ID
Q9UKM7
Alternative Names
MANA-ER
Function
Involved in glycoprotein quality control targeting of misfolded glycoproteins for degradation. It primarily trims a single alpha-1,2-linked mannose residue from Man(9)GlcNAc(2) to produce Man(8)GlcNAc(2), but at high enzyme concentrations, as found in the ER quality control compartment (ERQC), it further trims the carbohydrates to Man(5-6)GlcNAc(2).
Biological Process
Endoplasmic reticulum mannose trimmingManual Assertion Based On ExperimentIMP:ParkinsonsUK-UCL
Mannose trimming involved in glycoprotein ERAD pathwayManual Assertion Based On ExperimentIMP:ParkinsonsUK-UCL
N-glycan processingManual Assertion Based On ExperimentIBA:GO_Central
Oligosaccharide metabolic processManual Assertion Based On ExperimentTAS:ProtInc
Protein alpha-1,2-demannosylationManual Assertion Based On ExperimentIDA:ParkinsonsUK-UCL
Protein glycosylationIEA:UniProtKB-UniPathway
Trimming of first mannose on A branchTAS:Reactome
Trimming of second mannose on A branchTAS:Reactome
Trimming of terminal mannose on B branchManual Assertion Based On ExperimentIDA:ParkinsonsUK-UCL
Trimming of terminal mannose on C branchTAS:Reactome
Ubiquitin-dependent ERAD pathwayManual Assertion Based On ExperimentIMP:UniProtKB
Viral protein processingTAS:Reactome
Cellular Location
Endoplasmic reticulum membrane
Involvement in disease
Rafiq syndrome (RAFQS):
An autosomal recessive disorder characterized by variably impaired intellectual and motor development, a characteristic facial dysmorphism, truncal obesity, and hypotonia. The facial dysmorphism comprises prominent eyebrows with lateral thinning, downward-slanting palpebral fissures, bulbous tip of the nose, large ears, and a thin upper lip. Behavioral problems, including overeating, verbal and physical aggression, have been reported in some cases. Serum transferrin isoelectric focusing shows a type 2 pattern.
Topology
Cytoplasmic: 1-84
Helical: 85-105
Lumenal: 106-699

You, H., Zhang, N., Yu, T., Ma, L., Li, Q., Wang, X., ... & Tang, R. (2023). Hepatitis B virus X protein promotes MAN1B1 expression by enhancing stability of GRP78 via TRIM25 to facilitate hepatocarcinogenesis. British Journal of Cancer, 128(6), 992-1004.

Zhalsanova, I. Z., Ravzhaeva, E. G., Postrigan, A. E., Seitova, G. N., Zhigalina, D. I., Udalova, V. Y., ... & Skryabin, N. A. (2022). Case Report: Compound Heterozygous Variants of the MAN1B1 Gene in a Russian Patient with Rafiq Syndrome. International Journal of Molecular Sciences, 23(18), 10606.

Wang, X., Chen, B., Cao, Y., He, Y., & Chen, W. (2022). Identification of MAN1B1 as a novel marker for bladder cancer and its relationship with immune cell infiltration. Journal of oncology, 2022.

Kasapkara, C. S., Olgac, A., Kilic, M., Keldermans, L., Matthijs, G., & Jaeken, J. (2021). MAN1B1-CDG: Novel patients and novel variant. Journal of Pediatric Endocrinology and Metabolism, 34(9), 1207-1209.

Okamoto, N., Ohto, T., Enokizono, T., Wada, Y., Kohmoto, T., Imoto, I., ... & Suzuki, T. (2021). Siblings with MAN1B1-CDG showing novel biochemical profiles. Cells, 10(11), 3117.

Sakhi, S., Cholet, S., Wehbi, S., Isidor, B., Cogne, B., Vuillaumier-Barrot, S., ... & Bruneel, A. (2021). MAN1B1-CDG: Three new individuals and associated biochemical profiles. Molecular Genetics and Metabolism Reports, 28, 100775.

Kemme, L., Grüneberg, M., Reunert, J., Rust, S., Park, J., Westermann, C., ... & Marquardt, T. (2021). Translational balancing questioned: Unaltered glycosylation during disulfiram treatment in mannosyl‐oligosaccharide alpha‐1, 2‐mannnosidase‐congenital disorders of glycosylation (MAN1B1‐CDG). JIMD reports, 60(1), 42-55.

Okamoto, N., Ohto, T., Enokizono, T., Wada, Y., Kohmoto, T., Imoto, I., ... & Suzuki, T. (2021). Siblings with MAN1B1-CDG Showing Novel Biochemical Profiles. Cells 2021, 10, 3117.

Sun, A. H., Collette, J. R., & Sifers, R. N. (2020). The cytoplasmic tail of human mannosidase Man1b1 contributes to catalysis-independent quality control of misfolded alpha1-antitrypsin. Proceedings of the National Academy of Sciences, 117(40), 24825-24836.

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

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