Anti -ARSA, CT (ARSA, Arylsulfatase A, Cerebroside-sulfatase, Arylsulfatase A component B, Arylsulfatase A component C)

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

Chromosome: 22; NC_000022.10 (51061182..51066601, complement). Location: 22q13.33

Monoclonal

IgG2a

498CT24.1.2

Mouse

Human

Affinity Purified
Purified by Protein G affinity chromatography.

Supplied as a liquid in PBS, pH 7.2, 0.1% sodium azide.

May be stored at 4 degree C for short-term only. For long-term storage, store at -20 degree C. Aliquots are stable for at least 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

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.

Antibodies; Abs to Disease Markers

ELISA (EL/EIA), Western Blot (WB)

Suitable for use in Western Blot, ELISA.
Dilution: ELISA: 1:1,000
Western Blot: 1:100~250

53,588 Da[Similar Products]

arylsulfatase A

arylsulfatase A; ASA; cerebroside-sulfatase

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: Hydrolase; Lipid Metabolism - sphingolipid; EC 3.1.6.8

Chromosomal Location of Human Ortholog: 22q13.33

Cellular Component: lysosomal lumen; extracellular space; 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: sphingolipid metabolic process; central nervous system development; 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

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