CAG46516.1
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
37,208 Da
NCBI Official Full Name
AGA, partial
NCBI Official Synonym Full Names
aspartylglucosaminidase
NCBI Protein Information
N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase; N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase; N4-(N-acetyl-beta-glucosaminyl)-L-asparagine amidase; aspartylglucosylamine deaspartylase; glycosylasparaginase
UniProt Protein Name
N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase
UniProt Synonym Protein Names
Aspartylglucosaminidase; Glycosylasparaginase; N4-(N-acetyl-beta-glucosaminyl)-L-asparagine amidaseCleaved into the following 2 chains:Glycosylasparaginase alpha chain; Glycosylasparaginase beta chain
UniProt Entry Name
ASPG_HUMAN
NCBI Summary for AGA
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. Alternatively spliced transcript variants have been identified. [provided by RefSeq, Jan 2010]
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: EC 3.5.1.26; Glycan Metabolism - other glycan degradation; Hydrolase
Chromosomal Location of Human Ortholog: 4q34.3
Cellular Component: endoplasmic reticulum; lysosome
Molecular Function: peptidase activity; protein self-association; N4-(beta-N-acetylglucosaminyl)-L-asparaginase activity
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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