Aspartylglucosaminidase

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Aspartylglucosaminidase
Protein AGA PDB 1apy.png
PDB rendering based on 1apy.
Available structures
PDB Ortholog search: PDBe, RCSB
Identifiers
Symbols AGA ; AGU; ASRG; GA
External IDs OMIM613228 MGI104873 HomoloGene13 GeneCards: AGA Gene
EC number 3.5.1.26
RNA expression pattern
PBB GE AGA 204333 s at tn.png
PBB GE AGA 204332 s at tn.png
PBB GE AGA 216064 s at tn.png
More reference expression data
Orthologs
Species Human Mouse
Entrez 175 11593
Ensembl ENSG00000038002 ENSMUSG00000031521
UniProt P20933 Q64191
RefSeq (mRNA) NM_000027 NM_001005847
RefSeq (protein) NP_000018 NP_001005847
Location (UCSC) Chr 4:
178.35 – 178.36 Mb
Chr 8:
53.51 – 53.52 Mb
PubMed search [1] [2]

N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase is an enzyme that in humans is encoded by the AGA gene.[1]

Aspartylglucosaminidase is an amidohydrolase enzyme 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.[1]

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