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Rabbit Anti-ARSA Recombinant Antibody (1) (CBMAB-A3676-YC)

Provided herein is a Rabbit monoclonal antibody against Mouse Arylsulfatase A. The antibody can be used for immunoassay techniques, such as ELISA, WB.
See all ARSA antibodies

Summary

Host Animal
Rabbit
Specificity
Mouse
Clone
1
Antibody Isotype
IgG
Application
ELISA, WB

Basic Information

Immunogen
Recombinant Mouse Arylsulfatase A/ARSA protein.
Specificity
Mouse
Antibody Isotype
IgG
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
WB1:500-1:2,000

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, pH 7.4
Preservative
None
Concentration
Batch dependent
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
Arylsulfatase A
Introduction
ARSA hydrolyzes cerebroside sulfate to cerebroside and sulfate. Defects in this gene lead to metachromatic leucodystrophy (MLD), a progressive demyelination disease which results in a variety of neurological symptoms and ultimately death.
Entrez Gene ID
11883
UniProt ID
P50428
Alternative Names
Arylsulfatase A; Cerebroside-Sulfatase; ASA; Metachromatic Leucodystrophy; EC 3.1.6.8; EC 3.1.6; MLD;
Function
Hydrolyzes cerebroside sulfate.
Biological Process
Glycosphingolipid metabolic process Source: Reactome
Neutrophil degranulation Source: Reactome
Cellular Location
Lysosome; Endoplasmic reticulum
Involvement in disease
Metachromatic leukodystrophy (MLD): An autosomal recessive disease caused by abnormal intralysosomal accumulation of cerebroside-3-sulfate in central and peripheral nervous systems, as well as other organs. MLD is clinically characterized by leukodystrophy, progressive demyelination and a variety of neurological symptoms, including gait disturbances, ataxias, optical atrophy, dementia, seizures, and spastic tetraparesis. Decreased arylsulfatase A activity is detected in urine, leukocytes, and fibroblasts of affected individuals. Several forms of the disease can be distinguished according to the age at onset and disease severity: late infantile, juvenile and adult forms, partial cerebroside sulfate deficiency, and pseudoarylsulfatase A deficiency. Individuals with pseudoarylsulfatase A deficiency have low arylsulfatase A activity but lack neurological manifestations and are apparently healthy.
Multiple sulfatase deficiency (MSD): The protein represented in this entry is involved in disease pathogenesis. Arylsulfatase A activity is impaired in multiple sulfatase deficiency due to mutations in SUMF1 (PubMed:15146462). SUMF1 mutations result in defective post-translational modification of ARSA at residue Cys-69 that is not converted to 3-oxoalanine (PubMed:7628016). A clinically and biochemically heterogeneous disorder caused by the simultaneous impairment of all sulfatases, due to defective post-translational modification and activation. It combines features of individual sulfatase deficiencies such as metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay.
PTM
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. This post-translational modification is severely defective in multiple sulfatase deficiency (MSD).

Xu, L., Zhong, M., Wang, Y., Wang, Z., Song, J., Zhao, J., ... & Zheng, X. (2021). Case Report: Novel Arylsulfatase A (ARSA) Gene Mutations in a Patient With Adult-Onset Metachromatic Leukodystrophy Misdiagnosed as Multiple Sclerosis. Frontiers in Neurology, 11, 1633.

Gómez-Torres, M. J., Huerta-Retamal, N., Robles-Gómez, L., Sáez-Espinosa, P., Aizpurua, J., Avilés, M., & Romero, A. (2021). Arylsulfatase A Remodeling during Human Sperm In Vitro Capacitation Using Field Emission Scanning Electron Microscopy (FE-SEM). Cells, 10(2), 222.

Yoo, H. S., Lee, J. S., Chung, S. J., Ye, B. S., Sohn, Y. H., Lee, S. J., & Lee, P. H. (2020). Changes in plasma arylsulfatase A level as a compensatory biomarker of early Parkinson’s disease. Scientific reports, 10(1), 1-6.

Ruan, Y., Zheng, R., Lin, Z. H., Gao, T., Xue, N. J., Cao, J., ... & Pu, J. L. (2020). Genetic analysis of arylsulfatase A (ARSA) in Chinese patients with Parkinson’s disease. Neuroscience Letters, 734, 135094.

Xu, L., Zhong, M., Wang, Y., Wang, Z., Song, J., Zhao, J., ... & Zheng, X. (2020). Case Report: Novel Arylsulfatase A (ARSA) Gene Mutations in a Patient With Adult-Onset Metachromatic Leukodystrophy Misdiagnosed as Multiple Sclerosis. Frontiers in Neurology, 11.

Hettiarachchi, D., & Dissanayake, V. H. W. (2020). Correction to: Three novel variants in the arylsulfatase A (ARSA) gene in patients with metachromatic leukodystrophy (MLD). BMC research notes, 13(1), 1-2.

Hettiarachchi, D., & Dissanayake, V. H. W. (2019). Three novel variants in the arylsulfatase A (ARSA) gene in patients with metachromatic leukodystrophy (MLD). BMC research notes, 12(1), 1-4.

Narayanan, D. L., Matta, D., Gupta, N., Kabra, M., Ranganath, P., Aggarwal, S., ... & Dalal, A. (2019). Spectrum of ARSA variations in Asian Indian patients with Arylsulfatase A deficient metachromatic leukodystrophy. Journal of human genetics, 64(4), 323-331.

Lee, J. S., Kanai, K., Suzuki, M., Kim, W. S., Yoo, H. S., Fu, Y., ... & Lee, S. J. (2019). Arylsulfatase A, a genetic modifier of Parkinson’s disease, is an α-synuclein chaperone. Brain, 142(9), 2845-2859.

Böhringer, J., Santer, R., Schumacher, N., Gieseke, F., Cornils, K., Pechan, M., ... & Müller, I. (2017). Enzymatic characterization of novel arylsulfatase a variants using human arylsulfatase A‐deficient immortalized mesenchymal stromal cells. Human mutation, 38(11), 1511-1520.

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

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