AAAS (aladin WD repeat nucleoporin)

2010-03-01  

Identity

HGNC
LOCATION
12q13.13
LOCUSID
ALIAS
AAA,AAASb,ADRACALA,ADRACALIN,ALADIN,GL003
FUSION GENES

Other Information

Locus ID:

NCBI: 8086
MIM: 605378
HGNC: 13666
Ensembl: ENSG00000094914

Variants:

dbSNP: 8086
ClinVar: 8086
TCGA: ENSG00000094914
COSMIC: AAAS

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000094914ENST00000209873Q9NRG9
ENSG00000094914ENST00000394384Q9NRG9
ENSG00000094914ENST00000547757F8VUB6
ENSG00000094914ENST00000548931H3BU82
ENSG00000094914ENST00000550286F8VZ44

Expression (GTEx)

0
50
100
150

Pathways

PathwaySourceExternal ID
RNA transportKEGGko03013
RNA transportKEGGhsa03013
Nuclear pore complexKEGGhsa_M00427
Nuclear pore complexKEGGM00427
Metabolism of proteinsREACTOMER-HSA-392499
Post-translational protein modificationREACTOMER-HSA-597592
SUMOylationREACTOMER-HSA-2990846
SUMO E3 ligases SUMOylate target proteinsREACTOMER-HSA-3108232
SUMOylation of DNA damage response and repair proteinsREACTOMER-HSA-3108214
DiseaseREACTOMER-HSA-1643685
Infectious diseaseREACTOMER-HSA-5663205
HIV InfectionREACTOMER-HSA-162906
HIV Life CycleREACTOMER-HSA-162587
Late Phase of HIV Life CycleREACTOMER-HSA-162599
Rev-mediated nuclear export of HIV RNAREACTOMER-HSA-165054
Host Interactions of HIV factorsREACTOMER-HSA-162909
Interactions of Vpr with host cellular proteinsREACTOMER-HSA-176033
Vpr-mediated nuclear import of PICsREACTOMER-HSA-180910
Interactions of Rev with host cellular proteinsREACTOMER-HSA-177243
Nuclear import of Rev proteinREACTOMER-HSA-180746
Influenza InfectionREACTOMER-HSA-168254
Influenza Life CycleREACTOMER-HSA-168255
Transport of Ribonucleoproteins into the Host NucleusREACTOMER-HSA-168271
Influenza Viral RNA Transcription and ReplicationREACTOMER-HSA-168273
Viral Messenger RNA SynthesisREACTOMER-HSA-168325
Export of Viral Ribonucleoproteins from NucleusREACTOMER-HSA-168274
NEP/NS2 Interacts with the Cellular Export MachineryREACTOMER-HSA-168333
Host Interactions with Influenza FactorsREACTOMER-HSA-168253
NS1 Mediated Effects on Host PathwaysREACTOMER-HSA-168276
Immune SystemREACTOMER-HSA-168256
Cytokine Signaling in Immune systemREACTOMER-HSA-1280215
Interferon SignalingREACTOMER-HSA-913531
Antiviral mechanism by IFN-stimulated genesREACTOMER-HSA-1169410
ISG15 antiviral mechanismREACTOMER-HSA-1169408
Gene ExpressionREACTOMER-HSA-74160
Processing of Capped Intron-Containing Pre-mRNAREACTOMER-HSA-72203
Transport of Mature Transcript to CytoplasmREACTOMER-HSA-72202
Transport of Mature mRNA derived from an Intron-Containing TranscriptREACTOMER-HSA-159236
Transport of Mature mRNAs Derived from Intronless TranscriptsREACTOMER-HSA-159234
Transport of the SLBP Dependant Mature mRNAREACTOMER-HSA-159230
Transport of the SLBP independent Mature mRNAREACTOMER-HSA-159227
Transport of Mature mRNA Derived from an Intronless TranscriptREACTOMER-HSA-159231
Gene Silencing by RNAREACTOMER-HSA-211000
Transcriptional regulation by small RNAsREACTOMER-HSA-5578749
Metabolism of non-coding RNAREACTOMER-HSA-194441
snRNP AssemblyREACTOMER-HSA-191859
Cell CycleREACTOMER-HSA-1640170
Cell Cycle, MitoticREACTOMER-HSA-69278
M PhaseREACTOMER-HSA-68886
Mitotic ProphaseREACTOMER-HSA-68875
Nuclear Envelope BreakdownREACTOMER-HSA-2980766
Nuclear Pore Complex (NPC) DisassemblyREACTOMER-HSA-3301854
Transmembrane transport of small moleculesREACTOMER-HSA-382551
SLC-mediated transmembrane transportREACTOMER-HSA-425407
Hexose transportREACTOMER-HSA-189200
Glucose transportREACTOMER-HSA-70153
Regulation of Glucokinase by Glucokinase Regulatory ProteinREACTOMER-HSA-170822
MetabolismREACTOMER-HSA-1430728
Metabolism of carbohydratesREACTOMER-HSA-71387
Cellular responses to stressREACTOMER-HSA-2262752
Cellular response to heat stressREACTOMER-HSA-3371556
Regulation of HSF1-mediated heat shock responseREACTOMER-HSA-3371453
tRNA processingREACTOMER-HSA-72306
tRNA processing in the nucleusREACTOMER-HSA-6784531
SUMOylation of RNA binding proteinsREACTOMER-HSA-4570464
SUMOylation of DNA replication proteinsREACTOMER-HSA-4615885

Protein levels (Protein atlas)

Not detected
Low
Medium
High

References

Pubmed IDYearTitleCitations
327002932021Triple A (Allgrove) syndrome due to AAAS gene mutation with a rare association of amyotrophy.1
327002932021Triple A (Allgrove) syndrome due to AAAS gene mutation with a rare association of amyotrophy.1
310714872019Homozygous deletion of the entire AAAS gene in a triple A syndrome patient.1
310714872019Homozygous deletion of the entire AAAS gene in a triple A syndrome patient.1
292376972018Triple-A syndrome: a wide spectrum of adrenal dysfunction.11
292559502018Clinical and genetic characterisation of a series of patients with triple A syndrome.8
298741942018Clinical heterogeneity and molecular profile of triple A syndrome: a study of seven cases.8
303819132018Triple A syndrome presenting as complicated hereditary spastic paraplegia.2
292376972018Triple-A syndrome: a wide spectrum of adrenal dysfunction.11
292559502018Clinical and genetic characterisation of a series of patients with triple A syndrome.8
298741942018Clinical heterogeneity and molecular profile of triple A syndrome: a study of seven cases.8
303819132018Triple A syndrome presenting as complicated hereditary spastic paraplegia.2
274148112016Splicing Defects in the AAAS Gene Leading to both Exon Skipping and Partial Intron Retention in a Tunisian Patient with Allgrove Syndrome.2
274148112016Splicing Defects in the AAAS Gene Leading to both Exon Skipping and Partial Intron Retention in a Tunisian Patient with Allgrove Syndrome.2
257815312015Triple A syndrome with a novel indel mutation in the AAAS gene and delayed puberty.1

Citation

Dessen P

AAAS (aladin WD repeat nucleoporin)

Atlas Genet Cytogenet Oncol Haematol. 2010-03-01

Online version: http://atlasgeneticsoncology.org/gene/51360/aaas