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Mouse Anti-ARSA Recombinant Antibody (4C10) (CBMAB-BR036LY)

The product is antibody recognizes ARSA. The antibody 4C10 immunoassay techniques such as: FC, IHC-P, WB.
See all ARSA antibodies

Summary

Host Animal
Mouse
Specificity
Human, Mouse, Rat
Clone
4C10
Antibody Isotype
IgG2a
Application
FC, IHC-P, WB

Basic Information

Immunogen
A synthetic peptide corresponding to a sequence at the C-terminus of human ARSA.
Specificity
Human, Mouse, Rat
Antibody Isotype
IgG2a
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
WB0.1-0.5 μg/ml
IHC-P0.5-1 μg/ml
FC1-3 μg/1x10^6 cells

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

Format
Lyophilized
Buffer
PBS, Trehalose
Preservative
0.05 mg sodium azide
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
Arylsulfatase A
Introduction
The protein encoded by this gene 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. Alternatively spliced transcript variants have been described for this gene. [provided by RefSeq, Dec 2010]
Entrez Gene ID
Human410
Mouse11883
Rat315222
UniProt ID
HumanP15289
MouseP50428
RatQ32KK2
Alternative Names
Arylsulfatase A; ASA; Cerebroside-sulfatase; Arylsulfatase A component B; Arylsulfatase A component C; ARSA
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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