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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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KANSL3
KAT8 regulatory NSL complex subunit 3
Chromosome 2 · 2q11.2
NCBI Gene: 55683Ensembl: ENSG00000114982.20HGNC: HGNC:25473UniProt: Q9P2N6
53PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Highly Constrained
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
histone acetyltransferase complexNSL complexnucleoplasmmitochondrionplacental retentionNeurodevelopmental disorderresponse to xenobiotic stimuluspoisoning
✦AI Summary

KANSL3 is a non-catalytic component of the NSL histone acetyltransferase complex that mediates histone H4 acetylation at lysine-5 and lysine-8 residues at transcription start sites, promoting transcription initiation 1. Beyond its chr2-remodeling role, KANSL3 exhibits diverse cellular functions. It is required to promote KAT8 association with mitochondrial DNA and regulates mitochondrial transcription 2. KANSL3 maintains nuclear architecture stability by enabling KAT8-mediated acetylation of lamin A/C; loss of KANSL3 results in nuclear abnormalities and genomic instability including chr2 3. During mitosis, KANSL3 relocates to the mitotic spindle and functions as a microtubule minus-end-binding protein essential for spindle assembly 4. Hepatocyte-specific KANSL3 deletion causes early-onset liver disease with biliary hyperplasia and fibrosis by disrupting hepatic metabolic transcriptional networks and hepatocyte differentiation 5. KANSL3 is essential for cancer cell fitness in triple-negative breast cancer and pediatric high-grade glioma 67. Neural depletion of KANSL3 triggers metabolic dysfunction leading to vascular pericyte activation and brain hemorrhaging 8.

Sources cited
1
KANSL3 is a non-catalytic component of the NSL complex that mediates histone H4 acetylation at Lys-5 and Lys-8 to promote transcription initiation
PMID: 20018852
2
KANSL3 is required for KAT8 binding to mitochondrial DNA and regulates mitochondrial transcription
PMID: 27768893
3
Loss of KANSL3 leads to nuclear abnormalities, genomic instability, and chromothripsis through impaired lamin A/C acetylation
PMID: 31576060
4
KANSL3 is a microtubule minus-end-binding protein essential for spindle assembly during mitosis
PMID: 26243146
5
Hepatocyte-specific KANSL3 deletion causes liver disease with biliary hyperplasia and fibrosis by disrupting hepatic metabolism and hepatocyte differentiation
PMID: 41044006
6
KANSL3 is essential for triple-negative breast cancer cell fitness
PMID: 35973998
7
KANSL3 is essential for pediatric high-grade glioma cancer stem cell growth
PMID: 37161535
8
Neural KANSL3 depletion causes metabolic dysfunction leading to vascular pericyte activation and brain hemorrhaging
PMID: 32541879
Disease Associationsⓘ20
placental retentionOpen Targets
0.28Weak
Neurodevelopmental disorderOpen Targets
0.15Weak
response to xenobiotic stimulusOpen Targets
0.02Suggestive
poisoningOpen Targets
0.02Suggestive
dilated cardiomyopathyOpen Targets
0.02Suggestive
retinal degenerationOpen Targets
0.02Suggestive
liver diseaseOpen Targets
0.02Suggestive
triple-negative breast cancerOpen Targets
0.01Suggestive
Abnormal sperm morphologyOpen Targets
0.01Suggestive
neoplasmOpen Targets
0.01Suggestive
retinitis pigmentosaOpen Targets
0.01Suggestive
infectionOpen Targets
0.01Suggestive
hepatocellular carcinomaOpen Targets
0.01Suggestive
Hepatic fibrosisOpen Targets
0.00Suggestive
non-Hodgkins lymphomaOpen Targets
0.00Suggestive
cancerOpen Targets
0.00Suggestive
retinitis pigmentosa 1Open Targets
0.00Suggestive
Miyoshi myopathyOpen Targets
0.00Suggestive
non-alcoholic steatohepatitisOpen Targets
0.00Suggestive
prostate cancerOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
MTRES1Shared pathway100%KAT5Protein interaction100%DMAP1Protein interaction100%MORF4L1Protein interaction100%RUVBL1Protein interaction100%MEAF6Protein interaction100%
Tissue Expression6 tissues
Bone Marrow
100%
Ovary
84%
Lung
70%
Heart
66%
Liver
55%
Brain
49%
Gene Interaction Network
Click a node to explore
KANSL3MTRES1KAT5DMAP1MORF4L1RUVBL1MEAF6
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q9P2N6
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.29Highly Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.19 [0.13–0.29]
RankingsWhere KANSL3 stands among ~20K protein-coding genes
  • #8,446of 20,598
    Most Researched53
  • #1,087of 17,882
    Most Constrained (LOEUF)0.29 · top 10%
Genes detectedKANSL3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
EPIKOL, a chromatin-focused CRISPR/Cas9-based screening platform, to identify cancer-specific epigenetic vulnerabilities.
PMID: 35973998
Cell Death Dis · 2022
1.00
2
The NSL complex maintains nuclear architecture stability via lamin A/C acetylation.
PMID: 31576060
Nat Cell Biol · 2019
0.90
3
MOF Acetyl Transferase Regulates Transcription and Respiration in Mitochondria.
PMID: 27768893
Cell · 2016
0.80
4
KANSL3 directs transcriptional programs essential for hepatic metabolism and differentiation.
PMID: 41044006
Life Sci Alliance · 2025
0.70
5
CRISPR-Cas9 knockout screen identifies novel treatment targets in childhood high-grade glioma.
PMID: 37161535
Clin Epigenetics · 2023
0.60