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ARSA (R291) polyclonal antibody

Product Specifications

Background

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.

CAS Number

9007-83-4

Structure Composition

Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Product Name Alternative

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

Swiss Prot

P15289

Reactivity

Human, Rat

Immunogen

Synthetic peptide, corresponding to amino acids 260-310 of Human ARSA.

Conjugation

Unconjugated

Applications

WB IHC

Dilution

WB: 1:500~1:1000 IHC: 1:50~1:200

Purity

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .

Modification

Unmodification

Molecular Weight

~ 54 kDa

Storage Conditions

Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Notes

For research use only, not for use in diagnostic procedure.

Specificity

ARSA (R291) polyclonal antibody detects endogenous levels of ARSA protein.

Applications Notes

Western blot (WB) analysis of ARSA (R291) pAb at 1:500 dilution Lane1:AML-12 whole cell lysate (40ug) Lane2:C6 whole cell lysate (40ug) Lane3:H1792 whole cell lysate (40ug) Lane4:HEK293T whole cell lysate (40ug) Lane5:PC3 whole cell lysate (40ug)

Host or Source

Rabbit

Available Sizes

Frequently Asked Questions

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