Genotype-specific differences in infertile men due to loss-of-function variants in M1AP or ZZS genes

Abstract Male infertility has been linked to M1AP. In mice, M1AP interacts with the ZZS proteins SHOC1/TEX11/SPO16, promoting DNA class I crossover formation during meiosis. To determine whether M1AP and ZZS proteins are involved in human male infertility by recombination failure, we screened for bi...

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Main Authors: Nadja Rotte, Jessica E M Dunleavy, Michelle D Runkel, Lina Bosse, Daniela Fietz, Adrian Pilatz, Johanna Kuss, Ann-Kristin Dicke, Sofia B Winge, Sara Di Persio, Christian Ruckert, Verena Nordhoff, Hans-Christian Schuppe, Kristian Almstrup, Sabine Kliesch, Nina Neuhaus, Birgit Stallmeyer, Moira K O’Bryan, Frank Tüttelmann, Corinna Friedrich
Format: Article
Language:English
Published: Springer Nature 2025-05-01
Series:EMBO Molecular Medicine
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Online Access:https://doi.org/10.1038/s44321-025-00244-0
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author Nadja Rotte
Jessica E M Dunleavy
Michelle D Runkel
Lina Bosse
Daniela Fietz
Adrian Pilatz
Johanna Kuss
Ann-Kristin Dicke
Sofia B Winge
Sara Di Persio
Christian Ruckert
Verena Nordhoff
Hans-Christian Schuppe
Kristian Almstrup
Sabine Kliesch
Nina Neuhaus
Birgit Stallmeyer
Moira K O’Bryan
Frank Tüttelmann
Corinna Friedrich
author_facet Nadja Rotte
Jessica E M Dunleavy
Michelle D Runkel
Lina Bosse
Daniela Fietz
Adrian Pilatz
Johanna Kuss
Ann-Kristin Dicke
Sofia B Winge
Sara Di Persio
Christian Ruckert
Verena Nordhoff
Hans-Christian Schuppe
Kristian Almstrup
Sabine Kliesch
Nina Neuhaus
Birgit Stallmeyer
Moira K O’Bryan
Frank Tüttelmann
Corinna Friedrich
author_sort Nadja Rotte
collection DOAJ
description Abstract Male infertility has been linked to M1AP. In mice, M1AP interacts with the ZZS proteins SHOC1/TEX11/SPO16, promoting DNA class I crossover formation during meiosis. To determine whether M1AP and ZZS proteins are involved in human male infertility by recombination failure, we screened for biallelic/hemizygous loss-of-function (LoF) variants in the human genes to select men with presumed protein deficiency (N = 24). After in-depth characterisation of testicular phenotypes, we identified gene-specific meiotic impairments: men with ZZS deficiency shared an early meiotic arrest. Men with LoF variants in M1AP exhibited a predominant metaphase I arrest with rare haploid round or even elongated spermatids. These differences were explained by different recombination failures: deficient ZZS function led to incorrect synapsis of homologous chromosomes, unrepaired DNA double-strand breaks, and incomplete recombination. Abolished M1AP led to a reduced number of recombination intermediates and class I crossover. Medically assisted reproduction resulted in the birth of a healthy child, offering the possibility of fatherhood to men with LoF variants in M1AP. Our study establishes M1AP as an important, but non-essential, functional enhancer in meiotic recombination.
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spelling doaj-art-0b8c2cc8d7d64646b5b1b4ca4ebb007f2025-08-20T03:21:02ZengSpringer NatureEMBO Molecular Medicine1757-46842025-05-011761417145110.1038/s44321-025-00244-0Genotype-specific differences in infertile men due to loss-of-function variants in M1AP or ZZS genesNadja Rotte0Jessica E M Dunleavy1Michelle D Runkel2Lina Bosse3Daniela Fietz4Adrian Pilatz5Johanna Kuss6Ann-Kristin Dicke7Sofia B Winge8Sara Di Persio9Christian Ruckert10Verena Nordhoff11Hans-Christian Schuppe12Kristian Almstrup13Sabine Kliesch14Nina Neuhaus15Birgit Stallmeyer16Moira K O’Bryan17Frank Tüttelmann18Corinna Friedrich19Centre of Medical Genetics, Institute of Reproductive Genetics, University of MünsterSchool of BioSciences and Bio21 Molecular Sciences and Biotechnology Institute, Faculty of Science, University of MelbourneCentre of Medical Genetics, Institute of Reproductive Genetics, University of MünsterCentre of Medical Genetics, Institute of Reproductive Genetics, University of MünsterInstitute of Veterinary Anatomy, Histology and Embryology, University of GießenClinic and Polyclinic for Urology, Paediatric Urology and Andrology, University Hospital GießenCentre of Medical Genetics, Institute of Reproductive Genetics, University of MünsterCentre of Medical Genetics, Institute of Reproductive Genetics, University of MünsterDepartment of Growth and Reproduction, University Hospital CopenhagenCentre of Reproductive Medicine and Andrology, University of MünsterDepartment of Medical Genetics, University Hospital MünsterCentre of Reproductive Medicine and Andrology, University of MünsterClinic and Polyclinic for Urology, Paediatric Urology and Andrology, University Hospital GießenDepartment of Growth and Reproduction, University Hospital CopenhagenCentre of Reproductive Medicine and Andrology, University of MünsterCentre of Reproductive Medicine and Andrology, University of MünsterCentre of Medical Genetics, Institute of Reproductive Genetics, University of MünsterSchool of BioSciences and Bio21 Molecular Sciences and Biotechnology Institute, Faculty of Science, University of MelbourneCentre of Medical Genetics, Institute of Reproductive Genetics, University of MünsterCentre of Medical Genetics, Institute of Reproductive Genetics, University of MünsterAbstract Male infertility has been linked to M1AP. In mice, M1AP interacts with the ZZS proteins SHOC1/TEX11/SPO16, promoting DNA class I crossover formation during meiosis. To determine whether M1AP and ZZS proteins are involved in human male infertility by recombination failure, we screened for biallelic/hemizygous loss-of-function (LoF) variants in the human genes to select men with presumed protein deficiency (N = 24). After in-depth characterisation of testicular phenotypes, we identified gene-specific meiotic impairments: men with ZZS deficiency shared an early meiotic arrest. Men with LoF variants in M1AP exhibited a predominant metaphase I arrest with rare haploid round or even elongated spermatids. These differences were explained by different recombination failures: deficient ZZS function led to incorrect synapsis of homologous chromosomes, unrepaired DNA double-strand breaks, and incomplete recombination. Abolished M1AP led to a reduced number of recombination intermediates and class I crossover. Medically assisted reproduction resulted in the birth of a healthy child, offering the possibility of fatherhood to men with LoF variants in M1AP. Our study establishes M1AP as an important, but non-essential, functional enhancer in meiotic recombination.https://doi.org/10.1038/s44321-025-00244-0M1APMeiosisCrossoverInfertilityRecombination
spellingShingle Nadja Rotte
Jessica E M Dunleavy
Michelle D Runkel
Lina Bosse
Daniela Fietz
Adrian Pilatz
Johanna Kuss
Ann-Kristin Dicke
Sofia B Winge
Sara Di Persio
Christian Ruckert
Verena Nordhoff
Hans-Christian Schuppe
Kristian Almstrup
Sabine Kliesch
Nina Neuhaus
Birgit Stallmeyer
Moira K O’Bryan
Frank Tüttelmann
Corinna Friedrich
Genotype-specific differences in infertile men due to loss-of-function variants in M1AP or ZZS genes
EMBO Molecular Medicine
M1AP
Meiosis
Crossover
Infertility
Recombination
title Genotype-specific differences in infertile men due to loss-of-function variants in M1AP or ZZS genes
title_full Genotype-specific differences in infertile men due to loss-of-function variants in M1AP or ZZS genes
title_fullStr Genotype-specific differences in infertile men due to loss-of-function variants in M1AP or ZZS genes
title_full_unstemmed Genotype-specific differences in infertile men due to loss-of-function variants in M1AP or ZZS genes
title_short Genotype-specific differences in infertile men due to loss-of-function variants in M1AP or ZZS genes
title_sort genotype specific differences in infertile men due to loss of function variants in m1ap or zzs genes
topic M1AP
Meiosis
Crossover
Infertility
Recombination
url https://doi.org/10.1038/s44321-025-00244-0
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