ASA (arylsulfatase A), also known as Cerebroside-sulfatase, ARSA or MLD, is a 507 amino acid lysosomal protein that belongs to the sulfatase family.
Functioning as a homodimer at a neutral pH and as a homooctamer at an acidic pH, ASA uses magnesium as a cofactor to catalyze the H2O-dependent hydrolysis of cerebroside 3-sulfate to cerebroside and sulfate.
Defects in the gene encoding ASA are a cause of metachromatic leukodystrophy (MLD), an intralysosomal storage disease that is characterized by ataxias, dementia, seizures, spastic tetraparesis and, ultimately, death.
Additionally, defects in ASA activity are associated with multiple sulfatase deficiency (MSD), a disorder that results in decreased activity of all known sulfatases and is generally characterized by metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay.
Applications:Suitable for use in Western Blot, Immunohistochemistry.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:1000Immunohistochemistry: 1:50-1:200 Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：50ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid PBS, 0.05% sodium azide, pH 7.2.
Specificity：Recognizes endogenous levels of ARSA. Species Crossreactivity: Human, mouse, rat
Isotype：IgG
Calc Applications Abbrev：IHC WB
Calc Crossreactivity：Hu Mo Rt
Immunogen：Synthetic peptide corresponding to amino acids 260-310 of Human ARSA.
