NAGA recombinant protein

NAGA Protein, Human, Recombinant (His Tag)

Cat. Num. AAA8121508

• Gene Names: NAGA; GALB; D22S674
• Purity: >95% as determined by SDS-PAGE
• Synonyms: NAGA; NAGA Protein; Human; Recombinant (His Tag); Human NAGA/Alpha-N-acetylgalactosaminidase Protein (His Tag); D22S674 Protein; GALB Protein; N-acetylgalactosaminidase; alpha-; NAGA recombinant protein

• Host: HEK293 Cells
• Purity/Purification: >95% as determined by SDS-PAGE
• Form/Format: Lyophilized from sterile PBS, pH7.4. Normally 5%-8% trehalose, mannitol and 0.01% Tween80 are added as protectants before lyophilization. Please refer to the specific buffer information in the hard copy of CoA.
• Sequence: Met1-Gln411

• Species: Human
• Endotoxin: <1.0EU per ug of the protein as determined by the LAL method
• Predicted N Terminal: Leu 18
• Tag: C-His
• Protein Construction: A DNA sequence encoding the human NAGA (NP_000253.1)(Met1-Gln411) was expressed with a polyhistidine tag at the C-terminus.
• Reconstitution: A hardcopy of COA with reconstitution instruction is sent along with the products. Please refer to it for detailed information.
• Preparation and Storage: Samples are stable for up to twelve months from date of receipt at -20 degree C to -80 degree C. Store it under sterile conditions at -20 degree C to -80 degree C. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.; In general, recombinant proteins are provided as lyophilized powder which are shipped at ambient temperature. Bulk packages of recombinant proteins are provided as frozen liquid.; They are shipped out with blue ice.

SDS-Page

Related Product Information for NAGA recombinant protein

Background: NAGA (Alpha-N-Acetylgalactosaminidase) is a Protein Coding gene. NAGA encodes the lysosomal enzyme alpha-N-acetylgalactosaminidase, which cleaves alpha-N-acetylgalactosaminyl moieties from glycoconjugates. It belongs to the glycosyl hydrolase 27 family. The antinociceptive effect of NAGA may involve the participation of endogenous opioid peptides and endogenous catecholamines. Normal alpha-NAGA is synthesized as a 52kDa precursor which matures to a 49kDa species through phosphorylation and carbohydrate trimming. Mutations in gene encoding alpha-NAGA cause a wide range of diseases, characterized by mild to severe clinical features. NAGA is widely expressed in the placenta, appendix, and other tissues. Diseases associated with NAGA include Kanzaki Disease and Schindler Disease, Type I.

NCBI and Uniprot Product Information

• NCBI GI #: 4557781
• NCBI GeneID: 4668
• NCBI Accession #: NP_000253.1
• NCBI GenBank Nucleotide #: NM_000262.2
• UniProt Accession #: P17050
• Molecular Weight: 46,565 Da
• NCBI Official Full Name: alpha-N-acetylgalactosaminidase
• NCBI Official Synonym Full Names: alpha-N-acetylgalactosaminidase
• NCBI Official Symbol: NAGA
• NCBI Official Synonym Symbols: GALB; D22S674
• NCBI Protein Information: alpha-N-acetylgalactosaminidase
• UniProt Protein Name: Alpha-N-acetylgalactosaminidase
• Protein Family: Alpha-N-acetylgalactosaminidase
• UniProt Gene Name: NAGA

NCBI Description

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 Description

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.

Protein type: EC 3.2.1.49; Glycan Metabolism - glycosphingolipid biosynthesis - globo series; Hydrolase

Chromosomal Location of Human Ortholog: 22q13.2

Cellular Component: cytoplasm

Molecular Function: alpha-galactosidase activity; alpha-N-acetylgalactosaminidase activity; protein homodimerization activity

Biological Process: carbohydrate catabolic process; glycolipid catabolic process; glycoside catabolic process

Disease: Kanzaki Disease; Schindler Disease, Type I