Product Name
ARSA, Recombinant Protein
Full Product Name
Recombinant Human ARSA Protein
Product Synonym Names
Arylsulfatase A; ASA; Cerebroside-sulfatase
Product Gene Name
ARSA recombinant protein
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Sequence
RPPNIVLIFA DDLGYGDLGC YGHPSSTTPN LDQLAAGGLR FTDFYVPVSL CTPSRAALLT GRLPVRMGMY PGVLVPSSRG GLPLEEVTVA EVLAARGYLT GMAGKWHLGV GPEGAFLPPH QGFHRFLGIP YSHDQGPCQN LTCFPPATPC DGGCDQGLVP IPLLANLSVE AQPPWLPGLE ARYMAFAHDL MADAQRQDRP FFLYYASHHT HYPQFSGQSF AERSGRGPFG DSLMELDAAV GTLMTAIGDL GLLEETLVIF TADNGPETMR MSRGGCSGLL RCGKGTTYEG GVREPALAFW PGHIAPGVTH ELASSLDLLP TLAALAGAPL PNVTLDGFDL SPLLLGTGKS PRQSLFFYPS YPDEVRGVFA VRSGKYKAHF FTQGSAHSDT TADPACHASS SLTAHEPPLL YDLSKDPGEN YNLLGGVAGA TPEVLQALKQ LQLLKAQLDA AVTFGPSQVA RGEDPALQIC CHPGCTPRPA CCHCPDPHAV DHHHHHH
3D Structure
ModBase 3D Structure for P15289
Purity/Purification
Greater than 95% as determined by reducing SDS-PAGE.
Form/Format
Supplied as a 0.2 muM filtered solution of 20mM Tris-HCl, 150mM NaCl, pH 7.5.
Endotoxin
Less than 0.1 ng/mug (1 IEU/mug) as determined by LAL test.
Directions for Use
Always centrifuge tubes before opening. Do not mix by vortex or pipetting. It is not recommended to reconstitute to a concentration less than 100 mug/ml. Dissolve the lyophilized protein in 1X PBS. Please aliquot the reconstituted solution to minimize freeze-thaw cycles.
Preparation and Storage
Lyophilized protein should be stored at -20 degree C, though stable at room temperature for 3 weeks. Reconstituted protein solution can be stored at 2-8 degree C for 2-7 days. Aliquots of reconstituted samples are stable at -20 degree C for 3 months.
Other Notes
Small volumes of ARSA recombinant protein 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.
Related Product Information for
ARSA recombinant protein
Recombinant Human ARSA Protein is produced by our mammalian expression system and the target gene encoding Arg19-Ala507 is expressed with a 6His tag at the C-terminus.
Applications Tested/Suitable for ARSA recombinant protein
ELISA (EIA),Western Blot (WB), SDS-PAGE, Mass Spectrometry (MS)
NCBI/Uniprot data below describe general gene information for ARSA. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000478.3
[Other Products]
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
44,881 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
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: EC 3.1.6.8; Hydrolase; Lipid Metabolism - sphingolipid
Chromosomal Location of Human Ortholog: 22q13.33
Cellular Component: endoplasmic reticulum lumen; lysosomal lumen; lysosome
Molecular Function: arylsulfatase activity; calcium ion binding; cerebroside-sulfatase activity; protein binding; sulfuric ester hydrolase activity
Biological Process: glycosphingolipid metabolic process; post-translational protein modification
Disease: Metachromatic Leukodystrophy
Research Articles on ARSA
1. First report of arylsulfatase A pseudodeficiency (ASA-PD) allele and haplotype frequencies in a North African population, reveals relatively high prevalence of the ASA-PD allele in the Tunisian population with an intermediate genetic structure between Africans, Middle-eastern and Europeans most probably linked to the particular geographic location of Tunisia and the several population incursions throughout its history
Precautions
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Disclaimer
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