Enzymes Recombinant Proteins

Mouse ARSA Recombinant Protein (RPPB1413)

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RPPB1413

Description

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Product Name:Mouse ARSA Recombinant Protein
Product Code:RPPB1413
Size:10µg
Species:Mouse
Target:ARSA
Synonyms:Arylsulfatase A, Arsa, As-2, AS-A, As2, ASA, AW212749, TISP73.
Source:Sf9 Insect cells
Physical Appearance:Sterile Filtered clear solution.
Formulation:ARSA protein solution (0.5mg/ml) containing Phosphate Buffered Saline (pH 7.4) and 10% glycerol.
Stability:Store at 4°C if entire vial will be used within 2-4 weeks.�Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.
Purity:Greater than 95% as determined by SDS-PAGE.
Amino Acid Sequence:ADPSPPNILL IFADDLGYGD LGSYGHPSST TPNLDQLAEG GLRFTDFYVP VSLCTPSRAA LLTGRLPVRSGMYPGVLGPS SQGGLPLEEVTLAEVLAARG YLTGMAGKWH LGVGPEGAFL PPHQGFHRFL GIPYSHDQGP CQNLTCFPPD IPCKGGCDQG LVPIPLLANL TVEAQPPWLPGLEARYVSFS RDLMADAQRQ GRPFFLYYAS HHTHYPQFSG QSFTKRSGRG PFGDSLMELD GAVGALMTTV GDLGLLEETL VIFTADNGPELMRMSNGGCSGLLRCGKGTT FEGGVREPAL VYWPGHITPG VTHELASSLD LLPTLAALTG APLPNVTLDG VDISPLLLGT GKSPRKSVFFYPPYPDEIHG VFAVRNGKYK AHFFTQGSAH SDTTSDPACH AANRLTAHEP PLLYDLSQDP GENYNVLESI EGVSPEALQA LKHIQLLKAQYDAAMTFGPS QIAKGEDPAL QICCQPSCTP HPVCCHCPGS QSHHHHHH

Arylsulfatase A (ARSA) hydrolyzes cerebrosidesulfate to cerebroside and sulfate. ARSA is inhibited by phosphate. The phosphate develops a covalent bond with the active site 3-oxoalanine. ARSA gene defects cause metachromatic leucodystrophy (MLD), a progressive demyelination disease which results in various neurological symptoms and ultimately death.

ARSA Mouse Recombinant produced in Sf9 is a single, glycosylated polypeptide chain containing 498 amino acids (18-506) and having a molecular mass of 53.2kDa (Molecular size on SDS-PAGE will appear at approximately 50-70kDa).ARSA Mouse is fused to an�9 amino acid His-Tag at C-terminus and purified by proprietary chromatographic techniques.

UniProt Protein Function: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: EC 3.1.6.8; Hydrolase; Lipid Metabolism - sphingolipidChromosomal Location of Human Ortholog: 15|15 E3Cellular Component: acrosome; cytoplasm; endosome; extracellular space; extrinsic to external side of plasma membrane; integral to membrane; lysosome; plasma membraneMolecular Function: arylsulfatase activity; calcium ion binding; sulfuric ester hydrolase activityBiological Process: autophagy; binding of sperm to zona pellucida; central nervous system development; response to estrogen stimulus; response to ethanol; response to methylmercury; response to nutrient; response to pH
UniProt Protein Details:
NCBI Summary:
UniProt Code:P50428
NCBI GenInfo Identifier:67972647
NCBI Gene ID:11883
NCBI Accession:NP_033843.2
UniProt Secondary Accession:P50428,Q9DC66
UniProt Related Accession:P50428
Molecular Weight:53,748 Da
NCBI Full Name:arylsulfatase A
NCBI Synonym Full Names:arylsulfatase A
NCBI Official Symbol:Arsa��
NCBI Official Synonym Symbols:ASA; As2; AS-A; As-2; TISP73; AW212749��
NCBI Protein Information:arylsulfatase A
UniProt Protein Name:Arylsulfatase A
UniProt Synonym Protein Names:Cerebroside-sulfatase
Protein Family:Arylsulfatase
UniProt Gene Name:Arsa��
UniProt Entry Name:
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Additional Information

Product Type:
Recombinant Protein
Species:
Mouse
Uniprot:
P50428
Research Area:
Enzymes
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