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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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CEP192
centrosomal protein 192
Chromosome 18 · 18p11.21
NCBI Gene: 55125Ensembl: ENSG00000101639.20HGNC: HGNC:25515UniProt: Q8TEP8
97PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
phosphatase bindingcentriolecentrosomeprocentrioletype 2 diabetes mellitusdiabetes mellitusintelligenceneurodegenerative disease
✦AI Summary

CEP192 is a critical centrosomal scaffold protein required for mitotic centrosome maturation and bipolar spindle assembly 1. As a major regulator of pericentriolar material (PCM) recruitment, CEP192 coordinates the assembly of protein complexes necessary for microtubule nucleation 12. During mitosis, CEP192 depletion prevents gamma-tubulin and pericentrin accumulation at centrosomes, resulting in failed spindle assembly despite normal centriole presence 1. Mechanistically, CEP192 functions as a centrosome-specific activating scaffold for Aurora kinase A (AURKA) and PLK1 3. CEP192 cooperates with CEP152 to recruit PLK4 to centrioles, enabling centriole duplication 4. The conserved Spd-2 domain adopts an extended cradle structure that promotes PCM scaffold assembly through PLK1-mediated phosphorylation and oligomerization 5. Clinically, CEP192 mutations cosegregate with cardiac diseases including left ventricular noncompaction and bicuspid aortic valve, acting as genetic modifiers in combination with MIB1 mutations 6. CEP192 degradation by TRIM37 ubiquitination modulates cancer cell sensitivity to PLK4 inhibition, with implications for neuroblastoma and breast cancer treatment 7. CEP192 mutations also associate with male infertility and Mosaic Variegated Aneuploidy 5.

Sources cited
1
CEP192 is essential for mitotic centrosome maturation and spindle assembly; its depletion prevents PCM protein accumulation while maintaining centrioles
PMID: 17980596
2
CEP192 forms a scaffolding for microtubule nucleation proteins that normally functions at centrosomes but can disperse when centrosomes are absent
PMID: 18469523
3
CEP192 wraps around Aurora-A, modulates its kinase activity, primes TPX2 interaction, and localizes mitotic Aurora-A activity at centrosomes
PMID: 39327527
4
CEP192 cooperates with CEP152 for PLK4 recruitment to centrioles and centriole duplication through negatively charged amino acid binding regions
PMID: 23641073
5
CEP192's conserved Spd-2 domain adopts an extended cradle structure that promotes PCM scaffold assembly; mutations are associated with male infertility and Mosaic Variegated Aneuploidy
PMID: 40106572
6
CEP192 variants cosegregate with left ventricular noncompaction and bicuspid aortic valve as genetic modifiers of MIB1 mutations
PMID: 36325906
7
TRIM37-mediated CEP192 degradation inhibits acentrosomal spindle assembly; CEP192 levels modulate cancer sensitivity to PLK4 inhibition
PMID: 32908304
8
CEP192 localizes in ninefold symmetry at centrosomes and contributes to the molecular arrangement that creates flexibility for PLK4 and procentriole positioning
PMID: 37707473
Disease Associationsⓘ20
type 2 diabetes mellitusOpen Targets
0.33Weak
diabetes mellitusOpen Targets
0.32Weak
intelligenceOpen Targets
0.17Weak
neurodegenerative diseaseOpen Targets
0.16Weak
schizophreniaOpen Targets
0.11Weak
hepatocellular carcinomaOpen Targets
0.08Suggestive
neoplasmOpen Targets
0.08Suggestive
gastric cancerOpen Targets
0.04Suggestive
inflammatory bowel diseaseOpen Targets
0.03Suggestive
self-injurious ideationOpen Targets
0.03Suggestive
Crohn's diseaseOpen Targets
0.03Suggestive
rheumatoid arthritisOpen Targets
0.03Suggestive
psoriasisOpen Targets
0.02Suggestive
sclerosing cholangitisOpen Targets
0.02Suggestive
ulcerative colitisOpen Targets
0.02Suggestive
infertilityOpen Targets
0.02Suggestive
hypothyroidismOpen Targets
0.02Suggestive
ankylosing spondylitisOpen Targets
0.02Suggestive
celiac diseaseOpen Targets
0.02Suggestive
liver cancerOpen Targets
0.02Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
TENT5CProtein interaction100%PLK1Protein interaction100%AURKAProtein interaction94%NINProtein interaction94%CEP295Protein interaction94%SEH1LProtein interaction92%
Tissue Expression6 tissues
Bone Marrow
100%
Ovary
32%
Heart
26%
Brain
22%
Liver
20%
Lung
18%
Gene Interaction Network
Click a node to explore
CEP192TENT5CPLK1AURKANINCEP295SEH1L
PROTEIN STRUCTURE
Preparing viewer…
PDB6FVI · 1.00 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.61LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.51 [0.43–0.61]
RankingsWhere CEP192 stands among ~20K protein-coding genes
  • #4,925of 20,598
    Most Researched97 · top quartile
  • #4,234of 17,882
    Most Constrained (LOEUF)0.61 · top quartile
Genes detectedCEP192
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
TRIM37 controls cancer-specific vulnerability to PLK4 inhibition.
PMID: 32908304
Nature · 2020
1.00
2
A Human Hereditary Cardiomyopathy Shares a Genetic Substrate With Bicuspid Aortic Valve.
PMID: 36325906
Circulation · 2023
0.90
3
Human Cep192 and Cep152 cooperate in Plk4 recruitment and centriole duplication.
PMID: 23641073
J Cell Sci · 2013
0.80
4
CEP192 localises mitotic Aurora-A activity by priming its interaction with TPX2.
PMID: 39327527
EMBO J · 2024
0.70
5
Centrosomal organization of Cep152 provides flexibility in Plk4 and procentriole positioning.
PMID: 37707473
J Cell Biol · 2023
0.60