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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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FIS1
fission, mitochondrial 1
Chromosome 7 · 7q22.1
NCBI Gene: 51024Ensembl: ENSG00000214253.10HGNC: HGNC:21689UniProt: Q9Y3D6
112PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
identical protein bindingnegative regulation of ATP metabolic processmitochondrial fissionprotein bindingneurodegenerative diseaseknee fracturetriple-negative breast cancerEhlers-Danlos syndrome
✦AI Summary

FIS1 (Fission, Mitochondrial 1) is a mitochondrial outer membrane protein that plays a key role in regulating mitochondrial dynamics and cellular homeostasis. Functionally, FIS1 mediates mitochondrial fragmentation and perinuclear clustering 1, playing a minor role in recruiting the fission mediator DRP1 to mitochondrial surfaces 2. FIS1 also regulates peroxisomal fission 3 and can induce cytochrome c release leading to apoptosis 1. Mechanistically, FIS1 controls distinct mitochondrial fission outcomes: peripheral fission regulated by FIS1 directs damaged mitochondria toward mitophagy, while midzone fission enables mitochondrial proliferation 4. FIS1 recruits the GAP protein TBC1D15 to mitochondria to regulate lysosomal RAB7 GTPase activity, thereby controlling mitochondria-lysosome contacts that mark fission sites 5. Post-translational modifications of FIS1 are functionally significant: lactylation promotes excessive mitochondrial fission and apoptosis in sepsis-induced kidney injury 6, while deSUMOylation preserves endothelial mitochondrial integrity against hypoxic stress 7. Clinically, dysregulated mitochondrial dynamics involving FIS1 contribute to multiple diseases including cardiac dysfunction, inflammatory bowel disease, pulmonary hypertension, and type 2 diabetes 8910.

Sources cited
1
FIS1 mediates mitochondrial fragmentation, perinuclear clustering, and can induce cytochrome c release leading to apoptosis
PMID: 12783892
2
FIS1 plays a minor role in recruiting DRP1 to the mitochondrial surface and regulating mitochondrial fission
PMID: 12861026
3
FIS1 mediates peroxisomal fission
PMID: 16107562
4
FIS1 regulates peripheral mitochondrial fission directed toward mitophagy, distinct from midzone fission that enables mitochondrial proliferation
PMID: 33953403
5
FIS1 recruits TBC1D15 to regulate RAB7 GTPase activity, controlling mitochondria-lysosome contacts that mark fission sites
PMID: 29364868
6
FIS1 lactylation at K20 promotes excessive mitochondrial fission, ATP depletion, and apoptosis in sepsis-induced acute kidney injury
PMID: 37479690
7
FIS1 deSUMOylation preserves endothelial mitochondrial integrity and protects against hypoxic pulmonary hypertension
PMID: 37589160
8
Dysregulation of FIS1-mediated mitochondrial fission contributes to cardiac hypertrophy, heart failure, ischemia/reperfusion injury, and cardiomyopathies
PMID: 37895224
9
Higher FIS1 levels are associated with decreased risk of ulcerative colitis, identifying FIS1 as a mitochondrial gene relevant to inflammatory bowel disease pathogenesis
PMID: 38103512
10
FIS1 controls mitochondrial fission and is implicated in type 2 diabetes pathophysiology involving mitochondrial dysfunction
PMID: 28131082
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.54Moderate
knee fractureOpen Targets
0.15Weak
triple-negative breast cancerOpen Targets
0.07Suggestive
Ehlers-Danlos syndromeOpen Targets
0.07Suggestive
neoplasmOpen Targets
0.07Suggestive
Crigler-Najjar syndrome type 2Open Targets
0.06Suggestive
maple syrup urine disease, mild variantOpen Targets
0.06Suggestive
sarcosinemiaOpen Targets
0.06Suggestive
schizophreniaOpen Targets
0.06Suggestive
acute myeloid leukemiaOpen Targets
0.06Suggestive
hepatocellular carcinomaOpen Targets
0.05Suggestive
viral meningitisOpen Targets
0.05Suggestive
transient familial neonatal hyperbilirubinemiaOpen Targets
0.05Suggestive
gliomaOpen Targets
0.05Suggestive
methylmalonic acidemia due to transcobalamin receptor defectOpen Targets
0.05Suggestive
Methylmalonic aciduria due to transcobalamin receptor defectOpen Targets
0.05Suggestive
Alzheimer diseaseOpen Targets
0.04Suggestive
chronic kidney diseaseOpen Targets
0.04Suggestive
gluthathione peroxidase deficiencyOpen Targets
0.04Suggestive
Rotor syndromeOpen Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
DNM1Protein interaction100%TBC1D17Protein interaction100%DENRProtein interaction100%PEX11AProtein interaction97%MARCHF5Protein interaction97%VDAC1Protein interaction96%
Tissue Expression6 tissues
Heart
100%
Liver
88%
Brain
62%
Lung
53%
Ovary
53%
Bone Marrow
47%
Gene Interaction Network
Click a node to explore
FIS1DNM1TBC1D17DENRPEX11AMARCHF5VDAC1
PROTEIN STRUCTURE
Preparing viewer…
PDB9AYD · 1.53 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.10LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.72 [0.49–1.10]
RankingsWhere FIS1 stands among ~20K protein-coding genes
  • #4,224of 20,598
    Most Researched112 · top quartile
  • #11,223of 17,882
    Most Constrained (LOEUF)1.10
Genes detectedFIS1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
PDHA1 hyperacetylation-mediated lactate overproduction promotes sepsis-induced acute kidney injury via Fis1 lactylation.
PMID: 37479690
Cell Death Dis · 2023
1.00
2
Distinct fission signatures predict mitochondrial degradation or biogenesis.
PMID: 33953403
Nature · 2021
0.90
3
Mitochondria-lysosome contacts regulate mitochondrial fission via RAB7 GTP hydrolysis.
PMID: 29364868
Nature · 2018
0.80
4
Role of Mitochondrial Dynamics in Heart Diseases.
PMID: 37895224
Genes (Basel) · 2023
0.70
5
Multi-omic insight into the molecular networks of mitochondrial dysfunction in the pathogenesis of inflammatory bowel disease.
PMID: 38103512
EBioMedicine · 2024
0.60