NP_000018.2
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
NCBI Official Full Name
N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase isoform 1 preproprotein
NCBI Official Synonym Full Names
aspartylglucosaminidase
NCBI Protein Information
N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase
UniProt Protein Name
N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase
UniProt Synonym Protein Names
Aspartylglucosaminidase; Glycosylasparaginase; N4-(N-acetyl-beta-glucosaminyl)-L-asparagine amidase
UniProt Entry Name
ASPG_HUMAN
NCBI Summary for AGA
This gene encodes a member of the N-terminal nucleophile (Ntn) hydrolase family of proteins. The encoded preproprotein is proteolytically processed to generate alpha and beta chains that comprise the mature enzyme. This enzyme is involved in the catabolism of N-linked oligosaccharides of glycoproteins. It cleaves asparagine from N-acetylglucosamines as one of the final steps in the lysosomal breakdown of glycoproteins. Mutations in this gene are associated with the lysosomal storage disease aspartylglycosaminuria that results in progressive neurodegeneration. Alternative splicing results in multiple transcript variants, at least one of which encodes an isoform that is subject to proteolytic processing. [provided by RefSeq, Nov 2015]
UniProt Comments for AGA
AGA: a catabolic enzyme, aspartylglucosaminidase is involved in the catabolism of N-linked oligosaccharides of glycoproteins. It cleaves asparagine from N-acetylglucosamines as one of the final steps in the lysosomal breakdown of glycoproteins. The lysosomal storage disease aspartylglycosaminuria is caused by a deficiency in the AGA enzyme.
Protein type: Hydrolase; EC 3.5.1.26; Glycan Metabolism - other glycan degradation
Chromosomal Location of Human Ortholog: 4q34.3
Cellular Component: endoplasmic reticulum; extracellular space; lysosome
Molecular Function: N4-(beta-N-acetylglucosaminyl)-L-asparaginase activity; peptidase activity; protein self-association
Biological Process: protein deglycosylation; protein maturation; proteolysis
Disease: Aspartylglucosaminuria
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Products associated with anti-AGA antibody
Pathways associated with anti-AGA antibody
Diseases associated with anti-AGA antibody
Organs/Tissues associated with anti-AGA antibody
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