Full Product Name
Rabbit ARSA Antibody
Product Gene Name
anti-ARSA antibody
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Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Chromosome Location
Chromosome: 22; NC_000022.10 (51061182..51066601, complement). Location: 22q13.33
3D Structure
ModBase 3D Structure for P15289
Species Reactivity
Human, mouse, rat
Form/Format
Phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol
Concentration
1 mg/ml (lot specific)
Other Notes
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.
Applications Tested/Suitable for anti-ARSA antibody
ELISA (EIA), Immunohistochemistry (IHC), Western Blot (WB)
NCBI/Uniprot data below describe general gene information for ARSA. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000478.3
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NCBI GenBank Nucleotide #
NM_000487.5
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UniProt Primary Accession #
P15289
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UniProt Secondary Accession #
Q6ICI5; Q96CJ0; B2RCA6; B7XD04; F8WCC8[Other Products]
UniProt Related Accession #
P15289[Other Products]
Molecular Weight
53,588 Da
NCBI Official Full Name
arylsulfatase A isoform a
NCBI Official Synonym Full Names
arylsulfatase A
NCBI Official Symbol
ARSA??[Similar Products]
NCBI Official Synonym Symbols
MLD
??[Similar Products]
NCBI Protein Information
arylsulfatase A; ASA; cerebroside-sulfatase
UniProt Protein Name
Arylsulfatase A
UniProt Synonym Protein Names
Cerebroside-sulfatase
Protein Family
Arylsulfatase
UniProt Gene Name
ARSA??[Similar Products]
UniProt Synonym Gene Names
ASA??[Similar Products]
UniProt Entry Name
ARSA_HUMAN
NCBI Summary for ARSA
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 Comments for ARSA
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: 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
Research Articles on ARSA
1. The interaction between SPAM1, ARSA and HSPA2 in a multimeric complex mediating sperm-egg interaction.
Precautions
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