AGA (aspartylglucosaminidase)

2003-05-01  

Identity

HGNC
LOCATION
4q34.3
LOCUSID
ALIAS
AGU,ASRG,GA
FUSION GENES

Other Information

Locus ID:

NCBI: 175
MIM: 613228
HGNC: 318
Ensembl: ENSG00000038002

Variants:

dbSNP: 175
ClinVar: 175
TCGA: ENSG00000038002
COSMIC: AGA

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000038002ENST00000264595P20933
ENSG00000038002ENST00000502310H0Y9C7
ENSG00000038002ENST00000510635H0Y8L9

Expression (GTEx)

0
5
10
15
20
25
30

Pathways

PathwaySourceExternal ID
Other glycan degradationKEGGko00511
Other glycan degradationKEGGhsa00511
LysosomeKEGGko04142
LysosomeKEGGhsa04142
Immune SystemREACTOMER-HSA-168256
Innate Immune SystemREACTOMER-HSA-168249
Neutrophil degranulationREACTOMER-HSA-6798695

Protein levels (Protein atlas)

Not detected
Low
Medium
High

References

Pubmed IDYearTitleCitations
309011252019The T99K variant of glycosylasparaginase shows a new structural mechanism of the genetic disease aspartylglucosaminuria.0
309011252019The T99K variant of glycosylasparaginase shows a new structural mechanism of the genetic disease aspartylglucosaminuria.0
299931272018Biochemical and structural insights into an allelic variant causing the lysosomal storage disorder - aspartylglucosaminuria.2
299931272018Biochemical and structural insights into an allelic variant causing the lysosomal storage disorder - aspartylglucosaminuria.2
284577192017Crystal structure of a mutant glycosylasparaginase shedding light on aspartylglycosaminuria-causing mechanism as well as on hydrolysis of non-chitobiose substrate.3
284577192017Crystal structure of a mutant glycosylasparaginase shedding light on aspartylglycosaminuria-causing mechanism as well as on hydrolysis of non-chitobiose substrate.3
274710122016Development of a model system for neuronal dysfunction in Fabry disease.9
274710122016Development of a model system for neuronal dysfunction in Fabry disease.9
232717572014Aspartylglucosaminuria: unusual neonatal presentation in Qatari twins with a novel aspartylglucosaminidase gene mutation and 3 new cases in a Turkish family.7
232717572014Aspartylglucosaminuria: unusual neonatal presentation in Qatari twins with a novel aspartylglucosaminidase gene mutation and 3 new cases in a Turkish family.7
218543562012Expanding targets for a metabolic therapy of cancer: L-asparaginase.41
218543562012Expanding targets for a metabolic therapy of cancer: L-asparaginase.41
191002472009Plasma lysosomal enzyme activities in congenital disorders of glycosylation, galactosemia and fructosemia.6
191002472009Plasma lysosomal enzyme activities in congenital disorders of glycosylation, galactosemia and fructosemia.6
189922242008Structural basis of aspartylglucosaminuria.6

Citation

Dessen P

AGA (aspartylglucosaminidase)

Atlas Genet Cytogenet Oncol Haematol. 2003-05-01

Online version: http://atlasgeneticsoncology.org/gene/593/aga