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26 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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CEP170
centrosomal protein 170
Chromosome 1 · 1q43
NCBI Gene: 9859Ensembl: ENSG00000143702.18HGNC: HGNC:28920UniProt: Q5SW79
184PubMed Papers
0Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Highly Constrained
RESEARCH IMPACT
Trending
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
centriolar subdistal appendagecentriolecentrosomeprotein binding
✦AI Summary

CEP170 (centrosomal protein 170) is a multifunctional centrosomal protein with critical roles in microtubule organization and cilia biology. Structurally, CEP170 localizes to centrosomes, centriolar subdistal appendages, and ciliary basal bodies, where it plays essential roles in centriole assembly and spindle organization 12. CEP170 promotes microtubule organization through interactions with regulatory proteins; Ccdc61 controls its centrosomal localization and regulates its binding to TANK binding kinase 1, which stabilizes microtubules 3. During cilia disassembly, CEP170 functions downstream of PI3K signaling via a PDK1/PKCι kinase axis and interacts with the KIF2A microtubule-depolymerizing kinesin 4. In dynein-2 assembly, CEP170 interacts with dynein-2 complexes to support intraflagellar transport and cilia function 5. CEP170 is essential for proper mitotic progression and spindle orientation; its knockdown causes spindle misorientation and impairs astral microtubule stability while affecting dynein/dynactin motor complex localization 6. Disease relevance includes associations with chr1 kidney disease as a validated causal protein 7 and esophageal cancer, where METTL3-mediated m6A modifications regulate CEP170 expression, promoting cell proliferation 6. CEP170 loss impairs cilia formation and hedgehog signaling 5, contributing to ciliopathy-like phenotypes 4.

Sources cited
1
CEP170 plays a role in microtubule organization
PMID: 15616186
2
CEP170 is required for centriole subdistal appendage assembly
PMID: 28422092
3
Ccdc61 controls CEP170 centrosomal localization and its interaction with TANK binding kinase 1, affecting microtubule stability and spindle assembly
PMID: 30354798
4
CEP170 functions downstream of PI3K signaling in cilia disassembly through PDK1/PKCι kinases and interacts with KIF2A kinesin
PMID: 39168978
5
CEP170 interacts with dynein-2 to support intraflagellar transport and cilia function
PMID: 38533689
6
CEP170 is critical for mitotic progression and spindle orientation; METTL3-mediated m6A modifications regulate CEP170 in esophageal cancer
PMID: 39708485
7
CEP170 is a validated causal protein associated with chronic kidney disease
PMID: 38898508
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
CNTLNProtein interaction97%PLK1Protein interaction91%NINProtein interaction90%MAPRE1Protein interaction90%KIFC3Protein interaction90%CNTRLProtein interaction88%
Tissue Expression

No tissue expression data available for this gene.

Gene Interaction Network
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CEP170CNTLNPLK1NINMAPRE1KIFC3CNTRL
PROTEIN STRUCTURE
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PDB4JON · 2.15 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.26Highly Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.18 [0.13–0.26]
RankingsWhere CEP170 stands among ~20K protein-coding genes
  • #2,341of 20,598
    Most Researched184 · top quartile
  • #846of 17,882
    Most Constrained (LOEUF)0.26 · top 5%
Genes detectedCEP170
Sources retrieved26 papers
Response time—
📄 Sources
26▼
1
Identification of novel therapeutic targets for chronic kidney disease and kidney function by integrating multi-omics proteome with transcriptome.
PMID: 38898508
Genome Med · 2024
1.00
2
METTL3-mediated CEP170 m6A modifications in spindle orientation and esophageal cancer cell proliferation.
PMID: 39708485
Int Immunopharmacol · 2025
0.90
3
A class I PI3K signalling network regulates primary cilia disassembly in normal physiology and disease.
PMID: 39168978
Nat Commun · 2024
0.80
4
Clinical and molecular characterization of 1q43q44 deletion and corpus callosum malformations: 2 new cases and literature review.
PMID: 36192753
Mol Cytogenet · 2022
0.70
5
Modeling microcephaly with cerebral organoids reveals a WDR62-CEP170-KIF2A pathway promoting cilium disassembly in neural progenitors.
PMID: 31197141
Nat Commun · 2019
0.68