Human AGA/N(4)-(beta-N-Acetylglucosaminyl)-L-Asparaginase ELISA Kit | AGA elisa kit
Human AGA/N(4)-(beta-N-Acetylglucosaminyl)-L-Asparaginase ELISA Kit
NCBI and Uniprot Product Information
NCBI Description
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 Description
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: Glycan Metabolism - other glycan degradation; EC 3.5.1.26; 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
Research Articles on AGA
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Product Notes
The Human AGA aga (Catalog #AAA2890198) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA2890198 ELISA Kit recognizes Human AGA. It is sometimes possible for the material contained within the vial of "AGA/N(4)-(beta-N-Acetylglucosaminyl)-L-Asparaginase, ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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