ARSA active protein

Recombinant ARSA

Cat. Num. AAA201091

• Gene Names: ARSA; ASA; MLD
• Applications: SDS-Page
• Purity: >95% by SDS-PAGE
• Synonyms: ARSA; Recombinant ARSA; Arylsulfatase A isoform a; ASA; MLD; ARSA active protein

• Host: Baculovirus
• Purity/Purification: >95% by SDS-PAGE
• Form/Format: Liquid; In Phosphate Buffered Saline (pH 7.4) Containing 10% Glycerol.
• Concentration: 0.25mg/ml (determined by Absorbance at 280nm) (varies by lot)
• Sequence Positions: 21-509aa
• Sequence: ADPRPPNIVL IFADDLGYGD LGCYGHPSST TPNLDQLAAG GLRFTDFYVP VSLCTPSRAA LLTGRLPVRM GMYPGVLVPS SRGGLPLEEV TVAEVLAARG YLTGMAGKWH LGVGPEGAFL PPHQGFHRFL GIPYSHDQGP CQNLTCFPPA TPCDGGCDQG LVPIPLLANL SVEAQPPWLP GLEARYMAFA HDLMADAQRQ DRPFFLYYAS HHTHYPQFSG QSFAERSGRG PFGDSLMELD AAVGTLMTAI GDLGLLEETL VIFTADNGPE TMRMSRGGCS GLLRCGKGTT YEGGVREPAL AFWPGHIAPG VTHELASSLD LLPTLAALAG APLPNVTLDG FDLSPLLLGT GKSPRQSLFF YPSYPDEVRG VFAVRTGKYK AHFFTQGSAH SDTTADPACH ASSSLTAHEP PLLYDLSKDP GENYNLLGGV AGATPEVLQA LKQLQLLKAQ LDAAVTFGPS QVARGEDPAL QICCHPGCTP RPACCHCPDP HAHHHHHH
• Sequence Length: 509

• Applicable Applications for ARSA active protein: Enzyme Activity, SDS-PAGE
• Species: Human
• Endotoxin: <1.0 EU per 1ug of protein (determined by LAL method)
• Tag: His-Tag
• Predicted N-Terminal: Ala
• Biological Activity: Specific activity is > 2,500 pmol/min/ug, and defined as the amount of enzyme that hydrolyze 4-Nitrocatechol at pH 5.0 at 37C.
• Preparation and Storage: Can be stored at 2 degree C to 8 degree C for 1 week. For long term storage, aliquot and store at -20 degree C to -80 degree C. Avoid repeated freezing and thawing cycles.

SDS-Page

(3ug by SDS-PAGE under reducing condition and visualized by coomassie blue stain )

Related Product Information for ARSA active protein

ARSA, also known as arylsulfatase A isoform a, is a member of the sulfatase family. It hydrolyzes cerebroside sulfate, namely cerebroside 3-sulfate. It is found in many tissues, but predominantly in myelin and kidney. This protein is activated by a posttranslational modification (PTM) with the oxidation of cysteine to formylglycine. It could be helpful in identification of lung and central nervous system cancer through the serum level of this protein. The ARSA deficiency results in metachromatic leukodystrophy (MLD), a lysosomal storage disease in the central and peripheral nervous systems with severe and progressive neurological symptoms. Recombinant human ARSA protein, fused to His-tag at C-terminus, was expressed in insect cell and purified by using conventional chromatography techniques.

Product Categories/Family for ARSA active protein

Immunology; Neuroscience; Enzymes & Proteases

References

Shahzad MA., et al, (2017) J Mol Neurosci. 63:84-90. Cesani M., et al, (2017) Hum Mutat. 37:16-27.

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: 53.0kDa (498aa); 50-70KDa (SDS-PAGE under reducing conditions)
• 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