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GeneE
10 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
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MEI1
meiotic double-stranded break formation protein 1
Chromosome 22 Β· 22q13.2
NCBI Gene: 150365Ensembl: ENSG00000167077.14HGNC: HGNC:28613UniProt: Q4G0I1
15PubMed Papers
21Diseases
0Drugs
9Pathogenic Variants
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
meiosis IHydatidiform Molecomplete hydatidiform moleneurodegenerative diseasemixed connective tissue disease
✦AI Summary

MEI1 (meiotic double-stranded break formation protein 1) is a vertebrate-specific gene essential for normal meiotic progression in both male and female gametes. The protein functions primarily in the initiation of programmed DNA double-strand breaks (DSBs) during meiotic prophase I, which are crucial for proper chromosome 22 and recombination 1. In MEI1-deficient mice, spermatocytes exhibit defective DSB formation, as evidenced by reduced histone H2AX phosphorylation levels comparable to SPO11-deficient animals, and failure of RAD51 protein loading onto meiotic chr22 1. Clinically, biallelic MEI1 mutations cause diverse reproductive phenotypes including male non-obstructive azoospermia due to meiotic arrest at the spermatocyte stage 2, and in females, recurrent hydatidiform moles, embryonic arrest, and recurrent implantation failure 3. Recent studies demonstrate that MEI1 mutations lead to aneuploid embryos with abnormal DNA methylation patterns 4. The gene shows testis-specific expression and encodes a protein without significant homology to known proteins, representing a unique vertebrate meiotic regulator 1. These findings establish MEI1 as a critical factor in human fertility, with mutations contributing to both male and female infertility through distinct but related meiotic defects.

Sources cited
1
MEI1 is required for normal meiotic chromosome synapsis and DSB formation, with mutants showing defective RAD51 loading and reduced H2AX phosphorylation
PMID: 14668445
2
Biallelic MEI1 variants cause meiosis arrest and non-obstructive azoospermia in males through defective DSB homeostasis
PMID: 36759719
3
MEI1 mutations cause recurrent hydatidiform moles, embryonic arrest, and recurrent implantation failure in females
PMID: 34037756
4
MEI1 mutations lead to aneuploid embryos with abnormal DNA methylation patterns
PMID: 38416203
5
MEI1 polymorphisms are associated with azoospermia due to meiotic arrest in some populations
PMID: 16683055
Disease Associationsβ“˜21
Hydatidiform MoleOpen Targets
0.61Moderate
complete hydatidiform moleOpen Targets
0.37Weak
neurodegenerative diseaseOpen Targets
0.32Weak
mixed connective tissue diseaseOpen Targets
0.19Weak
atrial fibrillationOpen Targets
0.11Weak
hypothyroidismOpen Targets
0.11Weak
azoospermiaOpen Targets
0.10Weak
skin cancerOpen Targets
0.08Suggestive
partial chromosome Y deletionOpen Targets
0.08Suggestive
spermatogenic failure 57Open Targets
0.07Suggestive
spermatogenic failure 50Open Targets
0.07Suggestive
spermatogenic failure 25Open Targets
0.07Suggestive
Female infertility due to fertilization defectOpen Targets
0.07Suggestive
spermatogenic failure, X-linked, 2Open Targets
0.07Suggestive
spermatogenic failure 61Open Targets
0.07Suggestive
spermatogenic failure 62Open Targets
0.07Suggestive
spermatogenic failure 88Open Targets
0.07Suggestive
spermatogenic failure 59Open Targets
0.07Suggestive
spermatogenic failure 60Open Targets
0.07Suggestive
spermatogenic failure 74Open Targets
0.07Suggestive
Hydatidiform mole, recurrent, 3UniProt
Pathogenic Variants9
NM_152513.4(MEI1):c.3170-1G>CLikely pathogenic
not provided|Hydatidiform mole, recurrent, 3
β˜…β˜…β˜†β˜†2026
NM_152513.4(MEI1):c.2339del (p.Pro780fs)Likely pathogenic
Hydatidiform mole, recurrent, 3
β˜…β˜†β˜†β˜†2026β†’ Residue 780
NM_152513.4(MEI1):c.2206del (p.Val736fs)Pathogenic
Hydatidiform mole, recurrent, 3|not provided
β˜…β˜†β˜†β˜†2025β†’ Residue 736
NM_152513.4(MEI1):c.3772G>T (p.Asp1258Tyr)Likely pathogenic
Hydatidiform mole, recurrent, 3|MEI1-related disorder
β˜…β˜†β˜†β˜†2022β†’ Residue 1258
NM_152513.4(MEI1):c.1000_1003del (p.Ser334fs)Pathogenic
not provided
β˜…β˜†β˜†β˜†2022β†’ Residue 334
NM_152513.4(MEI1):c.1097-1G>CLikely pathogenic
not provided
β˜…β˜†β˜†β˜†2022
NM_152513.4(MEI1):c.1538+1G>ALikely pathogenic
not provided
β˜…β˜†β˜†β˜†2022
NM_152513.4(MEI1):c.3452G>A (p.Trp1151Ter)Likely pathogenic
Hydatidiform mole, recurrent, 3
β˜…β˜†β˜†β˜†2019β†’ Residue 1151
NM_152513.4(MEI1):c.1196+1G>APathogenic
Hydatidiform mole, recurrent, 3
β˜†β˜†β˜†β˜†2019
View on ClinVar β†—
Related Genes
REC114Protein interaction99%TOP6BLProtein interaction91%ANKRD31Protein interaction78%IHO1Protein interaction78%SPO11Protein interaction77%MORC1Protein interaction77%
Tissue Expression6 tissues
Bone Marrow
100%
Lung
18%
Liver
5%
Ovary
5%
Brain
3%
Heart
3%
Gene Interaction Network
Click a node to explore
MEI1REC114TOP6BLANKRD31IHO1SPO11MORC1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted Β· UniProt Q4G0I1
View on AlphaFold β†—
Constraintβ“˜
LOEUFβ“˜
0.85LoF Tolerant
pLIβ“˜
0.00Tolerant
Observed/Expected LoF0.71 [0.60–0.85]
RankingsWhere MEI1 stands among ~20K protein-coding genes
  • #15,623of 20,598
    Most Researched15
  • #3,006of 5,498
    Most Pathogenic Variants9
  • #7,429of 17,882
    Most Constrained (LOEUF)0.85
Genes detectedMEI1
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
Polymorphic alleles of the human MEI1 gene are associated with human azoospermia by meiotic arrest.
PMID: 16683055
J Hum Genet Β· 2006
1.00
2
Novel biallelic mutations in MEI1: expanding the phenotypic spectrum to human embryonic arrest and recurrent implantation failure.
PMID: 34037756
Hum Reprod Β· 2021
0.90
3
Whole-genome sequencing identifies new candidate genes for nonobstructive azoospermia.
PMID: 36017582
Andrology Β· 2022
0.80
4
Defects in meiosis I contribute to the genesis of androgenetic hydatidiform moles.
PMID: 39545410
J Clin Invest Β· 2024
0.70
5
Novel MEI1 mutations cause chromosomal and DNA methylation abnormalities leading to embryonicΒ arrest and implantation failure.
PMID: 38416203
Mol Genet Genomics Β· 2024
0.60