arylsulfatase A, ASA, Cerebroside-sulfatase

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

Monoclonal

IgG

Rabbit

Affinity purification

0.2 um filtered solution in PBS

Store at -20°C, Avoid freeze / thaw cycles.

Manufactured in an ISO 9001:2015 Certified Laboratory.

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.

Arylsulfatase A (ARSA) is synthesized as a 52KDa lysosomal enzyme. It is a member of the sulfatase family that is required for the lysosomal degradation of cerebroside-3-sulfate, a sphingolipid sulfate ester and a major constituent of the myelin sheet. Arylsulfatase A is activated by a required co- or posttranslational modification with the oxidation of cysteine to formylglycine. It has been shown that within the lysosomes, the ARSA dimers can oligomerize to an octamer in a pH-dependent manner. The ARSA deficiency leads to metachromatic leucodystrophy (MLD), a lysosomal storage disorder associated with severe and progressive demyelination in he central and peripheral nervous system. Additionally, the serum level of arylsulfatase A might be helpful in diagnosis of lung and central nervous system cancer.

Western Blot (WB)

WB 1:500-1:2000

Western Blot analysis of HepG2 using ARSA Monoclonal Antibody at dilution of 1:500

Calculated: 54kDa
Observed: 60kDa

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; Hydrolase; Lipid Metabolism - sphingolipid

Chromosomal Location of Human Ortholog: 22q13.33

Cellular Component: endoplasmic reticulum lumen; lysosomal lumen; lysosome

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

Biological Process: glycosphingolipid metabolic process; post-translational protein modification

Disease: Metachromatic Leukodystrophy

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