Arylsulfatase A; ASA; Cerebroside-sulfatase; Arylsulfatase A component B; Arylsulfatase A component C; ARSA

For Research Use Only. Not for use in diagnostic procedures.

Monoclonal

IgG2a

498CT24.1.2

Mouse

This ARSA antibody is generated from mice immunized with a KLH conjugated synthetic peptide between 408-439 amino acids from the C-terminal region of human ARSA.

Purified monoclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein G column, followed by dialysis against PBS.

Vial Concentration: 0.5 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-ARSA antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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.

Cancer; Cell Biology; Metabolism; Neuroscience

Western Blot (WB), ELISA (EIA)

WB~~1:100~250

ARSA Antibody (C-term) western blot analysis in Jurkat cell line lysates (35ug/lane).This demonstrates the ARSA antibody detected the ARSA protein (arrow).

53588

arylsulfatase A

arylsulfatase A

Arylsulfatase A

ASA??[Similar Products]

ARSA_HUMAN

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]

ARSA: Hydrolyzes cerebroside sulfate. Defects in ARSA are a cause of leukodystrophy metachromatic (MLD). MLD is a disease due to a lysosomal storage defect. It is characterized by intralysosomal storage of cerebroside-3-sulfate in neural and non-neural tissues, with a diffuse loss of myelin in the central nervous system. Progressive demyelination causes a variety of neurological symptoms, including gait disturbances, ataxias, optical atrophy, dementia, seizures, and spastic tetraparesis. Three forms of the disease can be distinguished according to the age at onset: late- infantile, juvenile and *****. Arylsulfatase A activity is defective in multiple sulfatase deficiency (MSD). 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. Arylsulfatase A activity is impaired in multiple sulfatase deficiency due to mutations in SUMF1. SUMF1 mutations result in defective post-translational modification of ARSA at residue Cys- 69 that is not converted to 3-oxoalanine. Belongs to the sulfatase family.

Protein type: EC 3.1.6.8; Lipid Metabolism - sphingolipid; Hydrolase

Chromosomal Location of Human Ortholog: 22q13.33

Cellular Component: extracellular space; lysosomal lumen; extrinsic to external side of plasma membrane; lysosome; endoplasmic reticulum lumen; integral to membrane; acrosome; endosome

Molecular Function: arylsulfatase activity; sulfuric ester hydrolase activity; calcium ion binding; cerebroside-sulfatase activity

Biological Process: central nervous system development; sphingolipid metabolic process; response to ethanol; cellular protein metabolic process; binding of sperm to zona pellucida; response to estrogen stimulus; response to methylmercury; autophagy; glycosphingolipid metabolic process; post-translational protein modification; response to nutrient; response to pH

Disease: Metachromatic Leukodystrophy

Cesani, M., et al. Hum. Mutat. 30 (10), E936-E945 (2009) : Matzner, U., et al. J. Biol. Chem. 284(14):9372-9381(2009) Bisgaard, A.M., et al. Clin. Genet. 75(2):175-179(2009) Lugowska, A., et al. Clin. Genet. 75(1):57-64(2009) Oshikawa, M., et al. Mol. Vis. 15, 482-494 (2009) :

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.