NP_032695.3
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
47,235 Da
NCBI Official Full Name
alpha-N-acetylgalactosaminidase isoform 1
NCBI Official Synonym Full Names
N-acetyl galactosaminidase, alpha
NCBI Protein Information
alpha-N-acetylgalactosaminidase
UniProt Protein Name
Alpha-N-acetylgalactosaminidase
UniProt Synonym Protein Names
Alpha-galactosidase B
UniProt Comments for NAGa
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: 15 E1|15 38.56 cM
Cellular Component: cytoplasm; lysosome
Molecular Function: alpha-galactosidase activity; alpha-N-acetylgalactosaminidase activity; catalytic activity; hydrolase activity; hydrolase activity, acting on glycosyl bonds; hydrolase activity, hydrolyzing O-glycosyl compounds; protein homodimerization activity
Biological Process: carbohydrate catabolic process; carbohydrate metabolic process; glycolipid catabolic process; glycoside catabolic process; glycosylceramide catabolic process; metabolic process; oligosaccharide metabolic process
Research Articles on NAGa
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Products associated with anti-NAGa antibody
Pathways associated with anti-NAGa antibody
Diseases associated with anti-NAGa antibody
Organs/Tissues associated with anti-NAGa antibody
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