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Mouse Anti-IDUA Recombinant Antibody (452619) (CBMAB-A4461-YC)

Provided herein is a Mouse monoclonal antibody against Human Iduronidase, Alpha-L-. The antibody can be used for immunoassay techniques, such as WB.
See all IDUA antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
452619
Antibody Isotype
IgG1
Application
WB

Basic Information

Immunogen
Mouse myeloma cell line NS0-derived recombinant human alpha-L-Iduronidase/IDUA, Ala26-Pro653 (Ala26Thr).
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
Lyophilized from PBS with trehalose
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
Iduronidase, Alpha-L-
Introduction
IDUA is an enzyme that hydrolyzes the terminal alpha-L-iduronic acid residues of two glycosaminoglycans, dermatan sulfate and heparan sulfate. This hydrolysis is required for the lysosomal degradation of these glycosaminoglycans. Mutations in this gene th
Entrez Gene ID
3425
UniProt ID
P35475
Alternative Names
Iduronidase
Biological Process
Chondroitin sulfate catabolic process Source: Reactome
Dermatan sulfate catabolic process Source: UniProtKB
Disaccharide metabolic process Source: ProtInc
Glycosaminoglycan catabolic process Source: Reactome
Heparin catabolic process Source: MGI
Cellular Location
Lysosome
Involvement in disease
Mucopolysaccharidosis 1H (MPS1H):
A severe form of mucopolysaccharidosis type 1, a rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. Patients with MPS1H usually present, within the first year of life, a combination of hepatosplenomegaly, skeletal deformities, corneal clouding and severe mental retardation. Obstructive airways disease, respiratory infection and cardiac complications usually result in death before 10 years of age.
Mucopolysaccharidosis 1H/S (MPS1H/S):
A form of mucopolysaccharidosis type 1, a rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. MPS1H/S represents an intermediate phenotype of the MPS1 clinical spectrum. It is characterized by relatively little neurological involvement, but most of the somatic symptoms described for severe MPS1 develop in the early to mid-teens, causing considerable loss of mobility.
Mucopolysaccharidosis 1S (MPS1S):
A mild form of mucopolysaccharidosis type 1, a rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. Patients with MPS1S may have little or no neurological involvement, normal stature and life span, but present development of joints stiffness, mild hepatosplenomegaly, aortic valve disease and corneal clouding.
PTM
N-glycosylation at Asn-372 contributes to substrate binding and is required for full enzymatic activity.

Lin, H. Y., Chang, S. Y., Teng, H. H., Wu, H. J., Li, H. Y., Cheng, C. C., ... & Cheng, W. C. (2023). Discovery of small-molecule protein stabilizers toward exogenous alpha-l-iduronidase to reduce the accumulated heparan sulfate in mucopolysaccharidosis type I cells. European Journal of Medicinal Chemistry, 247, 115005.

Ngiwsara, L., Sawangareetrakul, P., Wattanasirichaigoon, D., Tim-Aroon, T., Dejkhamron, P., Champattanachai, V., ... & Svasti, J. (2022). Effects of gentamicin inducing readthrough premature stop Codons: A study of alpha-L-iduronidase nonsense variants in COS-7 Cells. Biochemical and Biophysical Research Communications, 636, 147-154.

Al Zaabi, N. N., Sirajum, M., Al‐Wawi, M. Z., & Al Suwaiji, M. (2022). Alpha‐L‐iduronidase deficiency: A novel mutation resulting in severe early presentation of Mucopolysaccharidosis type I and literature review of the molecular basis. Clinical Case Reports, 10(5), e05904.

Langan, T. J., Jalal, K., Barczykowski, A. L., Carter, R. L., Stapleton, M., Orii, K., ... & Tomatsu, S. (2020). Development of a newborn screening tool for mucopolysaccharidosis type I based on bivariate normal limits: Using glycosaminoglycan and alpha‐L‐iduronidase determinations on dried blood spots to predict symptoms. JIMD reports, 52(1), 35-42.

Kamranjam, M., & Alaei, M. (2019). Mutation analysis of the IDUA gene in Iranian patients with mucopolysaccharidosis type 1: Identification of four novel mutations. Genetic Testing and Molecular Biomarkers, 23(8), 515-522.

Cardon, F., Pallisse, R., Bardor, M., Caron, A., Vanier, J., Ele Ekouna, J. P., ... & Guillet, M. (2019). Brassica rapa hairy root based expression system leads to the production of highly homogenous and reproducible profiles of recombinant human alpha‐L‐iduronidase. Plant Biotechnology Journal, 17(2), 505-516.

Li, Y., Tang, X., Meng, Y., Luo, G., & Yu, X. (2019). A novel compound mutation in alpha-L-iduronidase gene causes mucopolysaccharidosis type I. Journal of Genetics, 98, 1-4.

Hordeaux, J., Hinderer, C., Goode, T., Katz, N., Buza, E. L., Bell, P., ... & Wilson, J. M. (2018). Toxicology study of intra-cisterna magna adeno-associated virus 9 expressing human alpha-L-iduronidase in rhesus macaques. Molecular Therapy-Methods & Clinical Development, 10, 79-88.

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

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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