Rabbit anti-Human, Rat ARSA Polyclonal Antibody | anti-ARSA antibody

ARSA (R291) Polyclonal Antibody

Cat. Num. AAA3003386

• Gene Names: ARSA; ASA; MLD
• Reactivity: Human, Rat
• Applications: Western Blot, Immunohistochemistry
• Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Synonyms: ARSA; Polyclonal Antibody; ARSA (R291) Polyclonal Antibody; Arylsulfatase A; ASA; Cerebroside-sulfatase; Arylsulfatase A component B; Arylsulfatase A component C; anti-ARSA antibody

• Host: Rabbit
• Reactivity: Human, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: ARSA (R291) polyclonal antibody detects endogenous levels of ARSA protein.
• Purity/Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Form/Format: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
• Sequence Length: 509

• Applicable Applications for anti-ARSA antibody: Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: WB: 1:500-1:1000; IHC: 1:50-1:200
• Immunogen: Synthetic peptide, corresponding to amino acids 260-310 of Human ARSA.
• Preparation and Storage: Store at 4 degree C short term.; Aliquot and store at -20 degree C long term.; Avoid freeze-thaw cycles.

Western Blot (WB)

(Western Blot (WB) analysis of ARSA (R291) pAb at 1:500 dilutionLane1: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))

Related Product Information for anti-ARSA antibody

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.

NCBI and Uniprot Product Information

• NCBI GI #: 313569791
• NCBI GeneID: 410
• NCBI Accession #: NP_000478.3
• NCBI GenBank Nucleotide #: NP_000478.3
• UniProt Accession #: P15289
• Molecular Weight: ~ 54kDa
• NCBI Official Full Name: arylsulfatase A isoform a
• NCBI Official Synonym Full Names: arylsulfatase A
• NCBI Official Symbol: ARSA
• NCBI Official Synonym Symbols: ASA; MLD
• NCBI Protein Information: arylsulfatase A
• UniProt Protein Name: Arylsulfatase A
• Protein Family: Arylsulfatase
• UniProt Gene Name: ARSA
• UniProt Synonym Gene Names: ASA
• UniProt Entry Name: ARSA_HUMAN

NCBI Description

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]

Uniprot Description

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 adult. 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; EC 3.1.6.8; Lipid Metabolism - sphingolipid

Chromosomal Location of Human Ortholog: 22q13.33

Cellular Component: extracellular space; lysosomal lumen; extrinsic to external side of plasma membrane; endoplasmic reticulum lumen; lysosome; integral to membrane; acrosome; endosome

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

Biological Process: response to ethanol; central nervous system development; sphingolipid metabolic process; 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