Human NAGA Recombinant Protein (RPPB1994)

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SKU:
RPPB1994
Product type:
Recombinant Protein
Size:
5ug
Species:
Human
Target:
NAGA
Synonyms:
Alpha-N-acetylgalactosaminidase
Synonyms:
N-Acetylgalactosaminidase Alpha
Synonyms:
NAGA
Synonyms:
Alpha-galactosidase B
Source:
Sf9 Insect cells
Uniprot:
P17050
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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
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