EXOSC1 - exosome component 1 Gene

Also Known as p13; CSL4; SKI4; Csl4p; PCH1F; Ski4p; CGI-108

Species: Homo sapiens

Gene Type: protein coding
Gene ID: 51013

About EXOSC1

Cytogenetic location: 10q24.1 Genomic coordinates (GRCh38): 10:97,435,909-97,446,006 (from NCBI)

This gene has 11 transcripts (splice variants), 213 orthologues and is associated with 1 phenotype. Ubiquitous expression in lymph node (RPKM 9.3), urinary bladder (RPKM 7.6) and 25 other tissues.

Summary

This gene encodes a core component of the exosome. The mammalian exosome is required for rapid degradation of AU rich element-containing RNAs but not for poly(A) shortening. The association of this protein with the exosome is mediated by protein-protein interactions with ribosomal RNA-processing protein 42 and ribosomal RNA-processing protein 46. Alternative splicing of this gene results in multiple transcript variants. [provided by RefSeq, Jan 2016]

EXOSC1 Products (6)

mRNA Protein Name
NM_001318362.2 NP_001305291.1 exosome complex component CSL4 isoform b
NM_001318363.2 NP_001305292.1 exosome complex component CSL4 isoform c
NM_001318364.2 NP_001305293.1 exosome complex component CSL4 isoform d
NM_001318365.2 NP_001305294.1 exosome complex component CSL4 isoform e
NM_001318366.2 NP_001305295.1 exosome complex component CSL4 isoform e
NM_016046.5 NP_057130.1 exosome complex component CSL4 isoform a

Gene Ontology

  • Molecular Function
  • Biological Process
  • Cellular Component
Molecular Function GO Annotation Evidence References Source
NOT enables RNA exonuclease activity IDA
IDA: Inferred from direct assay
17174896 GOA
enables protein binding IPI
IPI: Inferred from physical interaction
11812149 GOA
Biological Process GO Annotation Evidence References Source
involved in RNA catabolic process IDA
IDA: Inferred from direct assay
17174896 GOA
involved in RNA processing IDA
IDA: Inferred from direct assay
17174896 GOA
Cellular Component GO Annotation Evidence References Source
located in cytosol IDA
IDA: Inferred from direct assay
20531386 GOA
part of exosome (RNase complex) IDA
IDA: Inferred from direct assay
20531389 GOA
located in nucleolus IDA
IDA: Inferred from direct assay
11812149 GOA
located in nucleus IDA
IDA: Inferred from direct assay
20531386 GOA
EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

EXOSC1 Protein Structure

ECR1_N

ECR1_N: Exosome complex exonuclease RRP4 N-terminal region (8 - 39)

EXOSC1

EXOSC1: Exosome component EXOSC1/CSL4 (64 - 135)

  • 0
  • 100
  • 195 a.a.
Protein Preferred Names Protein Names

exosome complex component CSL4

  • 3'-5' exoribonuclease CSL4 homolog

EXOSC1 Protein-protein interaction Information

Type
Protein Name Protein ID Interactor Interactor Species Interactor ID Detection Method References
Intra
EXOSC1 Q9Y3B2 REL Homo sapiens Q04864-2
Y2H Prey Pooling
32296183
Intra
EXOSC1 Q9Y3B2 REL Homo sapiens Q04864-2
Y2H Array
32296183
Intra
EXOSC1 Q9Y3B2 ZNF143 Homo sapiens P52747
Y2H Prey Pooling
32296183
Intra
EXOSC1 Q9Y3B2 ZNF143 Homo sapiens P52747
Y2H Array
32296183
Intra
EXOSC1 Q9Y3B2 EXOSC4 Homo sapiens Q9NPD3
Anti Tag CoIP
12788944
Intra
EXOSC1 Q9Y3B2 EXOSC4 Homo sapiens Q9NPD3
Y2H
15231747
Intra
EXOSC1 Q9Y3B2 EXOSC4 Homo sapiens Q9NPD3
GMS
25043052
Intra
EXOSC1 Q9Y3B2 EXOSC4 Homo sapiens Q9NPD3
Anti Tag CoIP
33961781
Intra
EXOSC1 Q9Y3B2 EXOSC4 Homo sapiens Q9NPD3
Anti Tag CoIP
26496610
Intra
EXOSC1 Q9Y3B2 EXOSC4 Homo sapiens Q9NPD3
Anti Tag CoIP
28514442
Intra
EXOSC1 Q9Y3B2 EXOSC7 Homo sapiens Q15024
GMS
25043052
Intra
EXOSC1 Q9Y3B2 EXOSC7 Homo sapiens Q15024
Y2H
12419256
Intra
EXOSC1 Q9Y3B2 EXOSC7 Homo sapiens Q15024
Anti Tag CoIP
26496610
Intra
EXOSC1 Q9Y3B2 EXOSC7 Homo sapiens Q15024
Y2H
11812149
Intra
EXOSC1 Q9Y3B2 EXOSC7 Homo sapiens Q15024
Anti Tag CoIP
33961781
Intra
EXOSC1 Q9Y3B2 EXOSC7 Homo sapiens Q15024
Anti Tag CoIP
12788944
Intra
EXOSC1 Q9Y3B2 EXOSC7 Homo sapiens Q15024
Anti Tag CoIP
28514442
Intra
EXOSC1 Q9Y3B2 EXOSC5 Homo sapiens Q9NQT4
Pull Down
11812149
Intra
EXOSC1 Q9Y3B2 EXOSC5 Homo sapiens Q9NQT4
Y2H
27107012
Intra
EXOSC1 Q9Y3B2 EXOSC5 Homo sapiens Q9NQT4
Validated Y2H
25416956
Intra
EXOSC1 Q9Y3B2 EXOSC5 Homo sapiens Q9NQT4
Anti Tag CoIP
26496610
Intra
EXOSC1 Q9Y3B2 EXOSC5 Homo sapiens Q9NQT4
Y2H Array
25416956
Intra
EXOSC1 Q9Y3B2 EXOSC5 Homo sapiens Q9NQT4
Anti Tag CoIP
33961781
Intra
EXOSC1 Q9Y3B2 EXOSC5 Homo sapiens Q9NQT4
Y2H
15231747
Intra
EXOSC1 Q9Y3B2 EXOSC5 Homo sapiens Q9NQT4
Y2H Prey Pooling
32296183
Intra
EXOSC1 Q9Y3B2 EXOSC5 Homo sapiens Q9NQT4
Y2H
11812149
Intra
EXOSC1 Q9Y3B2 EXOSC5 Homo sapiens Q9NQT4
Y2H Array
32296183
Intra
EXOSC1 Q9Y3B2 EXOSC5 Homo sapiens Q9NQT4
Anti Tag CoIP
28514442
Intra
EXOSC1 Q9Y3B2 EXOSC5 Homo sapiens Q9NQT4
MAPPIT
25416956
Intra
EXOSC1 Q9Y3B2 EXOSC5 Homo sapiens Q9NQT4
SLC
27107012
Intra
EXOSC1 Q9Y3B2 EXOSC5 Homo sapiens Q9NQT4
Validated Y2H
27107012
Intra
EXOSC1 Q9Y3B2 EXOSC5 Homo sapiens Q9NQT4
BFG-2H
27107012
Intra
EXOSC1 Q9Y3B2 EXOSC5 Homo sapiens Q9NQT4
GMS
25043052
Intra
EXOSC1 Q9Y3B2 EXOSC5 Homo sapiens Q9NQT4
Y2H
12419256
Intra
EXOSC1 Q9Y3B2 EXOSC8 Homo sapiens Q96B26
Y2H
15231747
Intra
EXOSC1 Q9Y3B2 EXOSC8 Homo sapiens Q96B26
Anti Tag CoIP
33961781
Intra
EXOSC1 Q9Y3B2 EXOSC8 Homo sapiens Q96B26
Y2H Array
32296183
Intra
EXOSC1 Q9Y3B2 EXOSC8 Homo sapiens Q96B26
Y2H Prey Pooling
32296183
Intra
EXOSC1 Q9Y3B2 EXOSC8 Homo sapiens Q96B26
GMS
25043052
Intra
EXOSC1 Q9Y3B2 EXOSC8 Homo sapiens Q96B26
Anti Tag CoIP
26496610
Intra
EXOSC1 Q9Y3B2 EXOSC8 Homo sapiens Q96B26
Anti Tag CoIP
28514442
Intra
EXOSC1 Q9Y3B2 TCF4 Homo sapiens P15884
Validated Y2H
25416956
Intra
EXOSC1 Q9Y3B2 TCF4 Homo sapiens P15884
Y2H Array
25416956
Intra
EXOSC1 Q9Y3B2 ZNF76 Homo sapiens P36508
Y2H Array
32296183
Intra
EXOSC1 Q9Y3B2 ZNF76 Homo sapiens P36508
Y2H Prey Pooling
32296183
Intra
EXOSC1 Q9Y3B2 LNX1 Homo sapiens Q8TBB1
Y2H Array
32296183
Intra
EXOSC1 Q9Y3B2 LNX1 Homo sapiens Q8TBB1
Y2H Prey Pooling
32296183
Intra
EXOSC1 Q9Y3B2 PAK5 Homo sapiens Q9P286
Validated Y2H
25416956
Intra
EXOSC1 Q9Y3B2 DDIT4L Homo sapiens Q96D03
Y2H Prey Pooling
32296183
Intra
EXOSC1 Q9Y3B2 DDIT4L Homo sapiens Q96D03
Y2H Array
32296183
Intra
EXOSC1 Q9Y3B2 ASB9 Homo sapiens Q96DX5
Y2H Prey Pooling
32296183
Intra
EXOSC1 Q9Y3B2 ASB9 Homo sapiens Q96DX5
Y2H Array
32296183
Intra
EXOSC1 Q9Y3B2 KCTD1 Homo sapiens Q719H9
Y2H Prey Pooling
25416956
Intra
EXOSC1 Q9Y3B2 KCTD1 Homo sapiens Q719H9
Validated Y2H
25416956
Intra
EXOSC1 Q9Y3B2 KCTD1 Homo sapiens Q719H9
Y2H Prey Pooling
32296183
Intra
EXOSC1 Q9Y3B2 KCTD1 Homo sapiens Q719H9
Y2H Array
25416956
Intra
EXOSC1 Q9Y3B2 KCTD1 Homo sapiens Q719H9
Y2H Array
32296183
Cross: Cross-species interaction Intra: Intraspecies interaction

Related Diseases

Diseases Alias
Pontocerebellar Hypoplasia, Type 1f

  • PCH1F

  • Pontocerebellar Hypoplasia Type 1f

  • Pontocerebellar Hypoplasia 1f
Pontocerebellar Hypoplasia

  • Pch

  • Congenital Pontocerebellar Hypoplasia

  • Opch

  • Hypoplasia, Pontocerebellar

  • Pontoneocerebellar Hypoplasia

  • Nonsyndromic Pontocerebellar Hypoplasia
Diamond-Blackfan Anemia 7

  • DBA7

  • Rpl11-Related Diamond-Blackfan Anemia

  • Anemia, Diamond-Blackfan, Type 7
Pontocerebellar Hypoplasia, Type 1c

  • PCH1C

  • Hypomyelination With Spinal Muscular Atrophy And Cerebellar Hypoplasia

  • Pontocerebellar Hypoplasia Type 1c

  • Pontocerebellar Hypoplasia 1c

  • Doid:0112334

  • Hypoplasia, Pontocerebellar, Type 1c
Pontocerebellar Hypoplasia, Type 1b

  • Pontocerebellar Hypoplasia Type 1b

  • PCH1B

  • Pontocerebellar Hypoplasia 1b

  • Hypoplasia, Pontocerebellar, Type 1b
Pontocerebellar Hypoplasia, Type 1d

  • PCH1D

  • Pontocerebellar Hypoplasia Type 1d

  • Pontocerebellar Hypoplasia 1d

  • Doid:0112323

  • Hypoplasia, Pontocerebellar, Type 1d
Pontocerebellar Hypoplasia, Type 1e

  • Pontocerebellar Hypoplasia Type 1

  • PCH1E

  • Norman Disease

  • Pch1

  • Pontocerebellar Hypoplasia With Anterior Horn Cell Disease

  • Pontocerebellar Hypoplasia With Infantile Spinal Muscular Atrophy

  • Pontocerebellar Hypoplasia Type 1e

  • Pontocerebellar Hypoplasia 1e

  • Doid:0112322

  • Doid:0112330
Trichohepatoenteric Syndrome 1

  • Trichohepatoenteric Syndrome

  • Syndromic Diarrhea

  • Tricho-Hepato-Enteric Syndrome

  • Sd/The

  • Syndromic Diarrhea/Tricho-Hepato-Enteric Syndrome

  • THES1

  • Phenotypic Diarrhea

  • Thes

  • Phenotypic Diarrhea Of Infancy

  • Diarrhea, Syndromic

  • Diarrhea, Fatal Infantile, With Trichorrhexis Nodosa

  • Intractable Diarrhea With Phenotypic Anomalies

  • Syndromatic Diarrhea

  • Fatal Infantile Diarrhea With Trichorrhexis Nodosa
Congenital Aphakia

  • Congenital Absence Of Lens

  • Aphakia, Congenital Primary

  • Agenesis Of Lens
Histrionic Personality Disorder
Diseases Alias
Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Related Agents

  • Pre-clinical Phase

Pre-clinical Phase

Bioactive Molecules (1)
Cat. No. Product Name
Effect
Purity
Rare Diseases
HY-RS04596 EXOSC1 Human Pre-designed siRNA Set A Pre-designed Set / Unkown

Orthologs Information

Species Symbol Source ID
Macaca mulatta EXOSC1 VGNC VGNC:72289
Mus musculus EXOSC1 MGD MGI:1913833
Bos taurus EXOSC1 VGNC VGNC:28655
Canis familiaris EXOSC1 VGNC VGNC:40521
Rattus norvegicus EXOSC1 RGD RGD:1591855
Others EXOSC1 NCBI