Human GUSB Recombinant Protein (RPPB1780)
- SKU:
- RPPB1780
- Product Type:
- Recombinant Protein
- Species:
- Human
- Uniprot:
- P08236
- Research Area:
- Enzymes
Description
| Product Name: | Human GUSB Recombinant Protein |
| Product Code: | RPPB1780 |
| Size: | 5µg |
| Species: | Human |
| Target: | GUSB |
| Synonyms: | GUSB, BG, MPS7, Glucuronidase Beta, EC 3.2.1.31, Beta-G1,Beta-D-Glucuronidase, Glucuronidase, Beta, Beta-Glucuronidasem. |
| Source: | Sf9 Insect cells |
| Physical Appearance: | Sterile Filtered colorless solution. |
| Formulation: | GUSB protein solution (0.25mg/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 90.0% as determined by SDS-PAGE. |
| Amino Acid Sequence: | LQGGMLYPQESPSRECKELD GLWSFRADFS DNRRRGFEEQ WYRRPLWESG PTVDMPVPSS FNDISQDWRL RHFVGWVWYEREVILPERWT QDLRTRVVLR IGSAHSYAIV WVNGVDTLEH EGGYLPFEAD ISNLVQVGPL PSRLRITIAINNTLTPTTLP PGTIQYLTDT SKYPKGYFVQ NTYFDFFNYA GLQRSVLLYT TPTTYIDDIT VTTSVEQDSGLVNYQISVKG SNLFKLEVRL LDAENKVVAN GTGTQGQLKV PGVSLWWPYL MHERPAYLYS LEVQLTAQTSLGPVSDFYTL PVGIRTVAVT KSQFLINGKP FYFHGVNKHE DADIRGKGFD WPLLVKDFNL LRWLGANAFRTSHYPYAEEV MQMCDRYGIV VIDECPGVGL ALPQFFNNVS LHHHMQVMEE VVRRDKNHPA VVMWSVANEPASHLESAGYY LKMVIAHTKS LDPSRPVTFV SNSNYAADKG APYVDVICLN SYYSWYHDYG HLELIQLQLATQFENWYKKY QKPIIQSEYG AETIAGFHQD PPLMFTEEYQ KSLLEQYHLG LDQKRRKYVV GELIWNFADFMTEQSPTRVL GNKKGIFTRQ RQPKSAAFLL RERYWKIANE TRYPHSVAKS QCLENSLFTH HHHHH |
| Biological Activity: | Specific activity is > 1600 pmol/min/ug and is definedas the amount of enzyme that hydrolyze 1.0 pmole of 4-Methylumbelliferone to 4-Methylum-belliferyl-Beta-D-glucosiduronic acid per minute at 37C and pH6.0. |
GlucuronidaseBeta (GUSB) is a lysosomal hydrolase which is taking part in the stepwisedegradation of glucuronic acid-containing glycosaminoglycans and plays a significantrole in the degradation of dermatan and keratin sulfates. GUSB is comprised ofheparin sulfate, chondroitin sulfate and hyaluronan.
GUSB Human Recombinant produced in Sf9 Baculovirus cells is a single, glycosylated polypeptide chain containing 635 amino acids (23-651a.a) and having a molecular mass of 73.4kDa (Molecular size on SDS-PAGE will appear at approximately 70-100kDa). GUSB is fused to a 6 amino acid His-tag at C-terminus & purified by proprietary chromatographic techniques.
| UniProt Protein Function: | GUSB: Plays an important role in the degradation of dermatan and keratan sulfates. Defects in GUSB are the cause of mucopolysaccharidosis type 7 (MPS7); also known as Sly syndrome. MPS7 is an autosomal recessive lysosomal storage disease characterized by inability to degrade glucuronic acid-containing glycosaminoglycans. The phenotype is highly variable, ranging from severe lethal hydrops fetalis to mild forms with survival into adulthood. Most patients with the intermediate phenotype show hepatomegaly, skeletal anomalies, coarse facies, and variable degrees of mental impairment. Mucopolysaccharidosis type 7 is associated with non- immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders. Belongs to the glycosyl hydrolase 2 family. 2 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:Glycan Metabolism - glycosaminoglycan degradation; Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; Hydrolase; Carbohydrate Metabolism - starch and sucrose; Carbohydrate Metabolism - pentose and glucuronate interconversions; Xenobiotic Metabolism - drug metabolism - other enzymes; EC 3.2.1.31 Chromosomal Location of Human Ortholog: 7q21.11 Cellular Component: lysosomal lumen; membrane; intracellular membrane-bound organelle Molecular Function:protein domain specific binding; beta-glucuronidase activity; receptor binding Biological Process: glycosaminoglycan catabolic process; glycosaminoglycan metabolic process; carbohydrate metabolic process; pathogenesis; hyaluronan metabolic process; hyaluronan catabolic process Disease: Mucopolysaccharidosis, Type Vii |
| NCBI Summary: | This gene encodes a hydrolase that degrades glycosaminoglycans, including heparan sulfate, dermatan sulfate, and chondroitin-4,6-sulfate. The enzyme forms a homotetramer that is localized to the lysosome. Mutations in this gene result in mucopolysaccharidosis type VII. Alternative splicing results in multiple transcript variants. There are many pseudogenes of this locus in the human genome.[provided by RefSeq, May 2014] |
| UniProt Code: | P08236 |
| NCBI GenInfo Identifier: | 146345377 |
| NCBI Gene ID: | 2990 |
| NCBI Accession: | P08236.2 |
| UniProt Secondary Accession: | P08236,Q549U0, Q96CL9, B4E1F6, E9PCV0, |
| UniProt Related Accession: | P08236 |
| Molecular Weight: | 651 |
| NCBI Full Name: | Beta-glucuronidase |
| NCBI Synonym Full Names: | glucuronidase, beta |
| NCBI Official Symbol: | GUSB�� |
| NCBI Official Synonym Symbols: | BG; MPS7�� |
| NCBI Protein Information: | beta-glucuronidase; beta-G1; beta-D-glucuronidase |
| UniProt Protein Name: | Beta-glucuronidase |
| UniProt Synonym Protein Names: | Beta-G1 |
| Protein Family: | Beta-glucuronidase |
| UniProt Gene Name: | GUSB�� |
| UniProt Entry Name: | BGLR_HUMAN |
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