Mouse Anti-IDS Recombinant Antibody (CBYY-I2021) (CBMAB-I3191-YY)

Name: Mouse Anti-IDS Recombinant Antibody (CBYY-I2021)
SKU: CBMAB-I3191-YY
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

CBYY-I2021

Application

WB, IHC, IF, FC

Immunogen

Full length human recombinant protein of human IDS(NP_000193)

Specificity

Human

Antibody Isotype

IgG2a

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, 50% glycerol

Preservative

0.02% sodium azide

Concentration

0.79 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.

More Infomation

Target

Full Name

Iduronate 2-Sulfatase

Introduction

This gene encodes a member of the sulfatase family of proteins. The encoded preproprotein is proteolytically processed to generate two polypeptide chains. This enzyme is involved in the lysosomal degradation of heparan sulfate and dermatan sulfate. Mutations in this gene are associated with the X-linked lysosomal storage disease mucopolysaccharidosis type II, also known as Hunter syndrome. Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed.

Entrez Gene ID

3423

UniProt ID

P22304

Alternative Names

Iduronate 2-Sulfatase

Function

Lysosomal enzyme involved in the degradation pathway of dermatan sulfate and heparan sulfate.

Biological Process

Glycosaminoglycan catabolic process Source: UniProtKB

Cellular Location

Lysosome

Involvement in disease

Mucopolysaccharidosis 2 (MPS2):
An X-linked lysosomal storage disease characterized by intracellular accumulation of heparan sulfate and dermatan sulfate and their excretion in urine. Most children with MPS2 have a severe form with early somatic abnormalities including skeletal deformities, hepatosplenomegaly, and progressive cardiopulmonary deterioration. A prominent feature is neurological damage that presents as developmental delay and hyperactivity but progresses to mental retardation and dementia. They die before 15 years of age, usually as a result of obstructive airway disease or cardiac failure. In contrast, those with a mild form of MPS2 may survive into adulthood, with attenuated somatic complications and often without mental retardation.

PTM

Synthesized as a 75-kDa precursor form in the endoplasmic reticulum (ER), and then processed by proteolytic cleavage through various intermediates to yield a 55-kDa mature form, with the release of an 18 kDa polypeptide.
The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity.

Badenetti, L., Manzoli, R., Trevisan, M., D’Avanzo, F., Tomanin, R., & Moro, E. (2023). A novel CRISPR/Cas9-based iduronate-2-sulfatase (IDS) knockout human neuronal cell line reveals earliest pathological changes. Scientific Reports, 13(1), 10289.

Mashima, R., Ohira, M., Okuyama, T., Onodera, M., & Takada, S. (2023). A novel mucopolysaccharidosis type II mouse model with an iduronate-2-sulfatase-P88L mutation. Scientific Reports, 13(1), 7865.

Gusarova, V. D., Smolov, M. A., Lyagoskin, I. V., Degterev, M. B., Rechetnik, E. V., Rodionov, A. V., ... & Shukurov, R. R. (2023). Characterization of a HIR-Fab-IDS, Novel Iduronate 2-Sulfatase Fusion Protein for the Treatment of Neuropathic Mucopolysaccharidosis Type II (Hunter Syndrome). BioDrugs, 37(3), 375-395.

Arguello, A., Meisner, R., Thomsen, E. R., Nguyen, H. N., Ravi, R., Simms, J., ... & Sanchez, P. E. (2021). Iduronate-2-sulfatase transport vehicle rescues behavioral and skeletal phenotypes in a mouse model of Hunter syndrome. JCI insight, 6(19).

Giugliani, R., Martins, A. M., So, S., Yamamoto, T., Yamaoka, M., Ikeda, T., ... & Sato, Y. (2021). Iduronate-2-sulfatase fused with anti-hTfR antibody, pabinafusp alfa, for MPS-II: A phase 2 trial in Brazil. Molecular Therapy, 29(7), 2378-2386.

Ohira, M., Kikuchi, E., Mizuta, S., Yoshida, N., Onodera, M., Nakanishi, M., ... & Mashima, R. (2021). Production of therapeutic iduronate‐2‐sulfatase enzyme with a novel single‐stranded RNA virus vector. Genes to Cells, 26(11), 891-904.

Okuyama, T., Eto, Y., Sakai, N., Minami, K., Yamamoto, T., Sonoda, H., ... & Sato, Y. (2019). Iduronate-2-sulfatase with anti-human transferrin receptor antibody for neuropathic mucopolysaccharidosis II: a phase 1/2 trial. Molecular Therapy, 27(2), 456-464.

Pimentel, N., Rodríguez‐Lopez, A., Díaz, S., Losada, J. C., Díaz‐Rincón, D. J., Cardona, C., ... & Barrera‐Avellaneda, L. A. (2018). Production and characterization of a human lysosomal recombinant iduronate‐2‐sulfatase produced in Pichia pastoris. Biotechnology and applied biochemistry, 65(5), 655-664.

Osaki, Y., Saito, A., Kanemoto, S., Kaneko, M., Matsuhisa, K., Asada, R., ... & Imaizumi, K. (2018). Shutdown of ER-associated degradation pathway rescues functions of mutant iduronate 2-sulfatase linked to mucopolysaccharidosis type II. Cell Death & Disease, 9(8), 808.

Hoshina, H., Shimada, Y., Higuchi, T., Kobayashi, H., Ida, H., & Ohashi, T. (2018). Chaperone effect of sulfated disaccharide from heparin on mutant iduronate-2-sulfatase in mucopolysaccharidosis type II. Molecular genetics and metabolism, 123(2), 118-122.

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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

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

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