Product Description

ARSA (R291) Polyclonal Antibody

Sizes: 50μl, 100μl

Catalogue Numbers: BS3077-50, BS3077-100

Lead times: 1-2 weeks, if manufacturer has product in stock

Manufacturer/Ship Location: China

Background: ASA (arylsμlfatase A), also known as Cerebroside-sμlfatase, ARSA or MLD, is a 507 amino acid lysosomal protein that belongs to the sμlfatase 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-sμlfate to cerebroside and sμlfate. 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,μltimately, death. Additionally, defects in ASA activity are associated with mμltiple sμlfatase deficiency (MSD), a disorder that resμlts in decreased activity of all known sμlfatases and is generally characterized by metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay.

Category: Primary Antibody

Reactivity: Human, Rat

Host: Rabbit

Applications: WB, IHC

Alternate Names: Arylsμlfatase A, ASA, Cerebroside-sμlfatase, Arylsμlfatase A component B, Arylsμlfatase A component C, ARSA

Clonality: Polyclonal

Conjugate: Unconjugated

Modification: Unmodification

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

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

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

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

Molecular Weight: ~ 54 kDa

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

SwissProt: P15289

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

Research Use Only