Human NAGA Recombinant Protein (RPPB1994)
- SKU:
- RPPB1994
- Product Type:
- Recombinant Protein
- Species:
- Human
- Uniprot:
- P17050
- Research Area:
- Enzymes
Description
| Product Name: | Human NAGA Recombinant Protein |
| Product Code: | RPPB1994 |
| Size: | 5µg |
| Species: | Human |
| Target: | NAGA |
| Synonyms: | Alpha-N-acetylgalactosaminidase, N-Acetylgalactosaminidase Alpha, NAGA, Alpha-galactosidase B, NAGA, D22S674, GALB. |
| Source: | Sf9 Insect cells |
| Physical Appearance: | Sterile Filtered clear solution. |
| Formulation: | NAGA protein solution (0.5mg/ml) containing Phosphate buffered saline (pH7.4), 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: | LDNGLLQTPP MGWLAWERFR CNINCDEDPK NCISEQLFME MADRMAQDGW RDMGYTYLNI DDCWIGGRDA SGRLMPDPKR FPHGIPFLAD YVHSLGLKLG IYADMGNFTC MGYPGTTLDK VVQDAQTFAE WKVDMLKLDG CFSTPEERAQ GYPKMAAALN ATGRPIAFSC SWPAYEGGLP PRVNYSLLAD ICNLWRNYDD IQDSWWSVLS ILNWFVEHQD ILQPVAGPGH WNDPDMLLIG NFGLSLEQSR AQMALWTVLA APLLMSTDLR TISAQNMDIL QNPLMIKINQ DPLGIQGRRI HKEKSLIEVY MRPLSNKASA LVFFSCRTDM PYRYHSSLGQ LNFTGSVIYE AQDVYSGDII SGLRDETNFT VIINPSGVVM WYLYPIKNLE MSQQHHHHHH |
N-AcetylgalactosaminidaseAlpha (NAGA) is a lysosomal exoglycosidase which removes terminalalpha-N-acetylgalactosamine residues from glycopeptides and glycolipids. NAGAis necessary for the breakdown of glycolipids.
NAGA Human Recombinant produced in Sf9 Baculovirus cells is a single, glycosylated polypeptide chain containing 400 amino acids (18-411) and having a molecular mass of 45.5kDa (Molecular size on SDS-PAGE will appear at approximately 40-57kDa).NAGA is fused to 6 amino acid His-Tag at C-terminus and purified by proprietary chromatographic techniques.
| UniProt Protein Function: | NAGA: Removes terminal alpha-N-acetylgalactosamine residues from glycolipids and glycopeptides. Required for the breakdown of glycolipids. Defects in NAGA are the cause of Schindler disease (SCHIND). Schindler disease is a form of NAGA deficiency characterized by early onset neuroaxonal dystrophy and neurological signs (convulsion during fever, epilepsy, psychomotor retardation and hypotonia). NAGA deficiency is typically classified in three main phenotypes: NAGA deficiency type I (Schindler disease or Schindler disease type I) with severe manifestations; NAGA deficiency type II (Kanzazi disease or Schindler disease type II) which is mild; NAGA deficiency type III (Schindler disease type III) characterized by mild-to-moderate neurologic manifestations. NAGA deficiency results in the increased urinary excretion of glycopeptides and oligosaccharides containing alpha-N-acetylgalactosaminyl moieties. Inheritance is autosomal recessive. Defects in NAGA are the cause of Kanzaki disease (KANZD); also known as NAGA deficiency type II or Schindler disease type II. Kanzaki disease is an autosomal recessive disorder characterized by late onset, angiokeratoma corporis diffusum and mild intellectual impairment. Belongs to the glycosyl hydrolase 27 family. |
| UniProt Protein Details: | Protein type:Glycan Metabolism - glycosphingolipid biosynthesis - globo series; Hydrolase; EC 3.2.1.49 Chromosomal Location of Human Ortholog: 22q11 Cellular Component: lysosome; cytoplasm Molecular Function:protein homodimerization activity; alpha-galactosidase activity; alpha-N-acetylgalactosaminidase activity Biological Process: glycolipid catabolic process; carbohydrate catabolic process; glycoside catabolic process; glycosylceramide catabolic process; oligosaccharide metabolic process Disease: Schindler Disease, Type I; Kanzaki Disease |
| NCBI Summary: | NAGA encodes the lysosomal enzyme alpha-N-acetylgalactosaminidase, which cleaves alpha-N-acetylgalactosaminyl moieties from glycoconjugates. Mutations in NAGA have been identified as the cause of Schindler disease types I and II (type II also known as Kanzaki disease). [provided by RefSeq, Jul 2008] |
| UniProt Code: | P17050 |
| NCBI GenInfo Identifier: | 4557781 |
| NCBI Gene ID: | 4668 |
| NCBI Accession: | NP_000253.1 |
| UniProt Related Accession: | P17050 |
| Molecular Weight: | 46,565 Da |
| NCBI Full Name: | alpha-N-acetylgalactosaminidase |
| NCBI Synonym Full Names: | N-acetylgalactosaminidase, alpha- |
| NCBI Official Symbol: | NAGA�� |
| NCBI Official Synonym Symbols: | GALB; D22S674�� |
| NCBI Protein Information: | alpha-N-acetylgalactosaminidase; alpha-galactosidase B; Acetylgalactosaminidase, alpha-N- (alpha-galactosidase B) |
| UniProt Protein Name: | Alpha-N-acetylgalactosaminidase |
| UniProt Synonym Protein Names: | Alpha-galactosidase B |
| Protein Family: | Alpha-N-acetylgalactosaminidase |
| UniProt Gene Name: | NAGA�� |
| UniProt Entry Name: | NAGAB_HUMAN |
Related Products
