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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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CEP89
centrosomal protein 89
Chromosome 19 · 19q13.11
NCBI Gene: 84902Ensembl: ENSG00000121289.19HGNC: HGNC:25907UniProt: Q96ST8
34PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
cilium assemblyprotein bindingcentrosomecytosolneurodegenerative diseasepreeclampsiagoutcystinuria
✦AI Summary

CEP89 (centrosomal protein 89) is a dual-function protein essential for both ciliogenesis and mitochondrial metabolism. As a distal appendage component, CEP89 plays a specialized role in primary cilium dynamics, specifically regulating ciliary disassembly rather than assembly 1. CEP89 functions in preciliary vesicle recruitment by interacting with myristoylated Neuronal Calcium Sensor-1 (NCS1), which directly captures preciliary vesicles at the distal appendage 2. The protein is recruited to distal appendages through CEP83 in a hierarchical assembly pathway and is phosphorylated by TTBK2 kinase 34. In mitochondrial metabolism, CEP89 localizes to the intermembrane space and is required for complex IV activity and proper assembly 5. Loss of CEP89 function causes isolated complex IV deficiency, intellectual disability, and neurological dysfunction in humans, while Drosophila knockdown results in mitochondrial dysfunction, altered synaptic transmission, and learning defects 5. Clinically, CEP89 overexpression and copy number gains are associated with poor prognosis in ovarian cancer, suggesting potential therapeutic relevance 6. The protein also modulates G0 cell cycle arrest capacity, linking it to therapy resistance mechanisms 7.

Sources cited
1
CEP89 specifically regulates ciliary disassembly and Aurora A kinase signaling
PMID: 39696441
2
CEP89 recruits NCS1 to distal appendages for preciliary vesicle capture
PMID: 39882855
3
CEP89 functions selectively in RAB34+ vesicle recruitment in hierarchical distal appendage assembly
PMID: 39882846
4
CEP89 is phosphorylated by TTBK2 kinase during ciliogenesis
PMID: 32129703
5
CEP89 localizes to mitochondrial intermembrane space and is required for complex IV activity and neuronal function
PMID: 23575228
6
CEP89 overexpression and copy number gains predict poor prognosis in ovarian cancer
PMID: 35285957
7
CEP89 modulates G0 cell cycle arrest capacity and therapy resistance
PMID: 37221612
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.37Weak
preeclampsiaOpen Targets
0.28Weak
goutOpen Targets
0.27Weak
cystinuriaOpen Targets
0.27Weak
VitiligoOpen Targets
0.22Weak
colorectal adenocarcinomaOpen Targets
0.21Weak
neoplasmOpen Targets
0.19Weak
lung adenocarcinomaOpen Targets
0.19Weak
anaplastic oligodendrogliomaOpen Targets
0.18Weak
bile duct carcinomaOpen Targets
0.18Weak
carcinoma of liver and intrahepatic biliary tractOpen Targets
0.18Weak
clear cell renal carcinomaOpen Targets
0.18Weak
Hodgkins lymphomaOpen Targets
0.18Weak
melanocytic neoplasmOpen Targets
0.18Weak
oral squamous cell carcinomaOpen Targets
0.18Weak
pancreatic ductal adenocarcinomaOpen Targets
0.18Weak
small cell lung carcinomaOpen Targets
0.18Weak
squamous cell lung carcinomaOpen Targets
0.18Weak
superficial spreading melanomaOpen Targets
0.18Weak
undifferentiated pleomorphic sarcomaOpen Targets
0.18Weak
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
ODF2Protein interaction98%CCP110Protein interaction98%CEP164Protein interaction98%NINProtein interaction98%RAB3IPProtein interaction89%RAB8AProtein interaction87%
Tissue Expression6 tissues
Brain
100%
Ovary
45%
Heart
26%
Lung
24%
Bone Marrow
18%
Liver
14%
Gene Interaction Network
Click a node to explore
CEP89ODF2CCP110CEP164NINRAB3IPRAB8A
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q96ST8
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.17LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.93 [0.75–1.17]
RankingsWhere CEP89 stands among ~20K protein-coding genes
  • #11,127of 20,598
    Most Researched34
  • #12,275of 17,882
    Most Constrained (LOEUF)1.17
Genes detectedCEP89
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Myristoylated Neuronal Calcium Sensor-1 captures the preciliary vesicle at distal appendages.
PMID: 39882855
Elife · 2025
1.00
2
A hierarchical pathway for assembly of the distal appendages that organize primary cilia.
PMID: 39882846
Elife · 2025
0.90
3
Distinct roles of centriole distal appendage proteins in ciliary assembly and disassembly.
PMID: 39696441
Cell Commun Signal · 2024
0.80
4
CEP89 is required for mitochondrial metabolism and neuronal function in man and fly.
PMID: 23575228
Hum Mol Genet · 2013
0.70
5
Copy number and expression of CEP89, a protein required for ciliogenesis, are increased and predict poor prognosis in patients with ovarian cancer.
PMID: 35285957
Cell Biochem Funct · 2022
0.60