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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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MTFP1
mitochondrial fission process 1
Chromosome 22 · 22q12.2
NCBI Gene: 51537Ensembl: ENSG00000242114.7HGNC: HGNC:26945UniProt: Q9UDX5
37PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingmitochondrial fissionmitochondrionmitochondrial inner membraneneoplasmhepatocellular carcinomagastric cancercancer
✦AI Summary

MTFP1 (mitochondrial fission process 1) is an inner mitochondrial membrane protein that negatively regulates mitochondrial fusion to control mitochondrial dynamics and cellular homeostasis 1. Mechanistically, MTFP1 inhibits inner membrane fusion and recruits DRP1 for fission, thereby isolating damaged membrane subdomains into small MTFP1-enriched mitochondria (SMEM) that undergo autophagic degradation 12. This quality control function is essential for maintaining mtDNA copy number and nucleoid recycling 1. MTFP1 expression is nutrient-regulated through mTORC1-mediated translation, linking environmental stimuli to mitochondrial fission and apoptosis; mTOR inhibition reduces MTFP1 levels, causing mitochondrial hyperfusion and apoptosis 3. PPA2 activates MTFP1-DNM1L fission signaling to control both mitochondrial proliferation and stress-induced mitophagy 4. MTFP1 is transcriptionally regulated by thyroid hormone during stem cell development 5 and by the transcription factor ZBTB48 6. Clinically, MTFP1 dysregulation associates with sarcopenia, where it correlates with muscle wasting severity and serves as a diagnostic biomarker 7. In prostate cancer, MTFP1 shows differential expression between tumor and normal tissues, suggesting potential biomarker utility 8. Loss of MTFP1 function impairs these quality control mechanisms and can trigger apoptosis.

Sources cited
1
MTFP1 negatively regulates inner mitochondrial membrane fusion, controls mitochondrial quality control, and maintains mtDNA levels through SMEM formation and autophagic degradation
PMID: 38851188
2
mTORC1 stimulates MTFP1 translation to control mitochondrial fission and cell survival; mTOR inhibition causes mitochondrial hyperfusion and apoptosis via reduced MTFP1 expression
PMID: 28918902
3
PPA2 activates MTFP1-DNM1L fission signaling to promote mitochondrial proliferation at midzone and mitophagy at periphery during stress conditions
PMID: 40873007
4
MISO recruits MTFP1 to inhibit fusion and promote SMEM formation; inner membrane stresses stabilize MISO to trigger MTFP1-dependent quality control
PMID: 41398051
5
MTFP1 is identified as a key regulator of mitochondrial dynamics in sarcopenia with positive correlation to muscle weight and strong diagnostic capability as a biomarker
PMID: 38042218
6
Thyroid hormone directly activates MTFP1 transcription via intronic T3-response element during intestinal stem cell development and proliferation
PMID: 32827515
7
ZBTB48 transcription factor regulates MTFP1 expression at both mRNA and protein levels in zebrafish
PMID: 39987415
8
MTFP1 shows differential expression between prostate tumor and normal tissues with potential biomarker value for prostate cancer
PMID: 40824549
Disease Associationsⓘ20
neoplasmOpen Targets
0.09Suggestive
hepatocellular carcinomaOpen Targets
0.08Suggestive
gastric cancerOpen Targets
0.06Suggestive
cancerOpen Targets
0.04Suggestive
Peptic ulcerOpen Targets
0.04Suggestive
oral squamous cell carcinomaOpen Targets
0.03Suggestive
squamous cell lung carcinomaOpen Targets
0.02Suggestive
prostate cancerOpen Targets
0.02Suggestive
heart failureOpen Targets
0.02Suggestive
Hepatic steatosisOpen Targets
0.02Suggestive
dilated cardiomyopathyOpen Targets
0.02Suggestive
lung adenocarcinomaOpen Targets
0.02Suggestive
sarcopeniaOpen Targets
0.02Suggestive
lung cancerOpen Targets
0.01Suggestive
metabolic diseaseOpen Targets
0.01Suggestive
acute kidney injuryOpen Targets
0.01Suggestive
endometrial carcinomaOpen Targets
0.01Suggestive
liver and intrahepatic bile duct neoplasmOpen Targets
0.01Suggestive
Neoplasm of the liverOpen Targets
0.01Suggestive
steatosisOpen Targets
0.01Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
DNM1LProtein interaction59%MTFR1LShared pathway50%MTFR1Shared pathway33%MTFR2Shared pathway33%GDAP1Shared pathway33%MFFShared pathway20%
Tissue Expression6 tissues
Liver
100%
Lung
59%
Brain
27%
Bone Marrow
20%
Ovary
18%
Heart
17%
Gene Interaction Network
Click a node to explore
MTFP1DNM1LMTFR1LMTFR1MTFR2GDAP1MFF
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q9UDX5
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.63LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.12 [0.78–1.63]
RankingsWhere MTFP1 stands among ~20K protein-coding genes
  • #10,654of 20,598
    Most Researched37
  • #15,785of 17,882
    Most Constrained (LOEUF)1.63
Genes detectedMTFP1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
MTFP1 controls mitochondrial fusion to regulate inner membrane quality control and maintain mtDNA levels.
PMID: 38851188
Cell · 2024
1.00
2
mTOR Controls Mitochondrial Dynamics and Cell Survival via MTFP1.
PMID: 28918902
Mol Cell · 2017
0.90
3
PPA2 activates MTFP1-DNM1L fission signaling to govern mitochondrial proliferation and mitophagy.
PMID: 40873007
Autophagy · 2026
0.80
4
Zbtb48 is a regulator of Mtfp1 expression in zebrafish.
PMID: 39987415
Commun Biol · 2025
0.70
5
MISO regulates mitochondrial dynamics and mtDNA homeostasis by establishing membrane subdomains.
PMID: 41398051
Nat Cell Biol · 2026
0.60