ARSA, Polyclonal Antibody

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

Polyclonal

Rabbit

Affinity purified

Liquid

100ug/100ul (lot specific)

This product is stable for several weeks at 4 degree C as an undiluted liquid. Dilute only prior to immediate use. For extended storage, aliquot contents and freeze at -20 degree C or below. Avoid cycles of freezing and thawing. Expiration date is one (1) year from date of receipt.

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.

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]

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

ELISA titer: 1:20,000-1:80,000
Western blot analysis: 1:1,000-1:2,000
Immunohistochemistry: 1:100-1:500
Optimal dilutions/concentrations should be determined by the end user.

44,881 Da

arylsulfatase A

arylsulfatase A; ASA; cerebroside-sulfatase

Arylsulfatase A

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: Lipid Metabolism - sphingolipid; EC 3.1.6.8; Hydrolase

Chromosomal Location of Human Ortholog: 22q13.33

Cellular Component: extracellular space; lysosomal lumen; extrinsic to external side of plasma membrane; endoplasmic reticulum lumen; lysosome; 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; cellular protein metabolic process; response to ethanol; 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

(1) Hayashi,T., Nakamura,M., Ichiba,M., et al. Adult-type metachromatic leukodystrophy with compound heterozygous ARSA mutations: a case report and phenotypic comparison with a previously reported case. Psychiatry Clin. Neurosci. 65 (1), 105-108 (2011)

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