EXOSC3 (exosome component 3)

2014-11-01  

Identity

HGNC
LOCATION
9p13.2
LOCUSID
ALIAS
CGI-102,PCH1B,RRP40,Rrp40p,bA3J10.7,hRrp-40,p10

Other Information

Locus ID:

NCBI: 51010
MIM: 606489
HGNC: 17944
Ensembl: ENSG00000107371

Variants:

dbSNP: 51010
ClinVar: 51010
TCGA: ENSG00000107371
COSMIC: EXOSC3

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000107371ENST00000327304Q9NQT5
ENSG00000107371ENST00000396521Q9NQT5
ENSG00000107371ENST00000465229Q9NQT5

Expression (GTEx)

0
5
10
15
20
25
30
35

Pathways

PathwaySourceExternal ID
RNA degradationKEGGko03018
RNA degradationKEGGhsa03018
Exosome, eukaryotesKEGGhsa_M00391
Exosome, eukaryotesKEGGM00391
Metabolism of proteinsREACTOMER-HSA-392499
Unfolded Protein Response (UPR)REACTOMER-HSA-381119
PERK regulates gene expressionREACTOMER-HSA-381042
ATF4 activates genesREACTOMER-HSA-380994
Gene ExpressionREACTOMER-HSA-74160
Deadenylation-dependent mRNA decayREACTOMER-HSA-429914
mRNA decay by 3' to 5' exoribonucleaseREACTOMER-HSA-429958
Regulation of mRNA stability by proteins that bind AU-rich elementsREACTOMER-HSA-450531
Butyrate Response Factor 1 (BRF1) binds and destabilizes mRNAREACTOMER-HSA-450385
Tristetraprolin (TTP, ZFP36) binds and destabilizes mRNAREACTOMER-HSA-450513
KSRP (KHSRP) binds and destabilizes mRNAREACTOMER-HSA-450604
rRNA processingREACTOMER-HSA-72312
Major pathway of rRNA processing in the nucleolus and cytosolREACTOMER-HSA-6791226
rRNA processing in the nucleus and cytosolREACTOMER-HSA-8868773

Protein levels (Protein atlas)

Not detected
Low
Medium
High

References

Pubmed IDYearTitleCitations
355223392022Atypical hemolytic uremic syndrome induced by SARS-CoV2 infection in infants with EXOSC3 mutation.4
355223392022Atypical hemolytic uremic syndrome induced by SARS-CoV2 infection in infants with EXOSC3 mutation.4
334620002021Two siblings with a novel variant of EXOSC3 extended phenotypic spectrum of pontocerebellar hypoplasia 1B to an exceptionally mild form.2
346260222021Aberrant expression of MYD88 via RNA-controlling CNOT4 and EXOSC3 in colonic mucosa impacts generation of colonic cancer.3
334620002021Two siblings with a novel variant of EXOSC3 extended phenotypic spectrum of pontocerebellar hypoplasia 1B to an exceptionally mild form.2
346260222021Aberrant expression of MYD88 via RNA-controlling CNOT4 and EXOSC3 in colonic mucosa impacts generation of colonic cancer.3
326450032020A Drosophila model of Pontocerebellar Hypoplasia reveals a critical role for the RNA exosome in neurons.10
326450032020A Drosophila model of Pontocerebellar Hypoplasia reveals a critical role for the RNA exosome in neurons.10
280532712017Mutations of EXOSC3/Rrp40p associated with neurological diseases impact ribosomal RNA processing functions of the exosome in S. cerevisiae.21
286875122017Recessive mutation in EXOSC3 associates with mitochondrial dysfunction and pontocerebellar hypoplasia.15
280532712017Mutations of EXOSC3/Rrp40p associated with neurological diseases impact ribosomal RNA processing functions of the exosome in S. cerevisiae.21
286875122017Recessive mutation in EXOSC3 associates with mitochondrial dysfunction and pontocerebellar hypoplasia.15
271931682016Altered RNA metabolism due to a homozygous RBM7 mutation in a patient with spinal motor neuropathy.26
271931682016Altered RNA metabolism due to a homozygous RBM7 mutation in a patient with spinal motor neuropathy.26
245242992014EXOSC3 mutations in pontocerebellar hypoplasia type 1: novel mutations and genotype-phenotype correlations.40

Citation

Dessen P

EXOSC3 (exosome component 3)

Atlas Genet Cytogenet Oncol Haematol. 2014-11-01

Online version: http://atlasgeneticsoncology.org/gene/62951/exosc3-(exosome-component-3)