Mouse ARSA Recombinant Protein (RPPB1412)
- SKU:
- RPPB1412
- Product Type:
- Recombinant Protein
- Species:
- Mouse
- Uniprot:
- P50428
- Research Area:
- Enzymes
Frequently bought together:
Description
| Product Name: | Mouse ARSA Recombinant Protein |
| Product Code: | RPPB1412 |
| Size: | 10µg |
| Species: | Mouse |
| Target: | ARSA |
| Synonyms: | ArylsulfataseA, ASA, Cerebroside-sulfatase. |
| Source: | Sf9 Insect cells |
| Physical Appearance: | Sterile Filtered clear solution. |
| Formulation: | ARSA protein solution (0.5mg/ml) containing PhosphateBuffered 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 IFADDLGYGDLGSYGHPSST TPNLDQLAEG GLRFTDFYVP VSLCTPSRAA LLTGRLPVRS GMYPGVLGPS SQGGLPLEEVTLAEVLAARG YLTGMAGKWH LGVGPEGAFL PPHQGFHRFL GIPYSHDQGP CQNLTCFPPD IPCKGGCDQGLVPIPLLANL TVEAQPPWLP GLEARYVSFS RDLMADAQRQ GRPFFLYYAS HHTHYPQFSG QSFTKRSGRGPFGDSLMELD GAVGALMTTV GDLGLLEETL VIFTADNGPE LMRMSNGGCS GLLRCGKGTT FEGGVREPALVYWPGHITPG VTHELASSLD LLPTLAALTG APLPNVTLDG VDISPLLLGT GKSPRKSVFF YPPYPDEIHGVFAVRNGKYK AHFFTQGSAH SDTTSDPACH AANRLTAHEP PLLYDLSQDP GENYNVLESI EGVSPEALQALKHIQLLKAQ YDAAMTFGPS 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 Baculovirus cells is a single, glycosylated polypeptide chain containing 498 amino acids (18-506 a.a.) and having a molecular mass of 53.2kDa (Molecular size on SDS-PAGE will appear at approximately 50-70kDa). ARSA is expressed with a 6 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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