Mutation in

genome-wide linkage molecular genetics

Journal

Journal of medical genetics
ISSN: 1468-6244
Titre abrégé: J Med Genet
Pays: England
ID NLM: 2985087R

Informations de publication

Date de publication:
05 Jun 2020
Historique:
received: 03 01 2020
revised: 01 04 2020
accepted: 08 04 2020
entrez: 7 6 2020
pubmed: 7 6 2020
medline: 7 6 2020
Statut: aheadofprint

Résumé

Oligoteratoasthenozoospermia (OTA) combines deteriorated quantity, morphology and motility of the sperm, resulting in male factor infertility. We used whole genome genotyping and exome sequencing to identify the mutation causing OTA in four men in a consanguineous Bedouin family. We expressed the normal and mutated proteins tagged with c-Myc at the carboxy termini by transfection with pCDNA3.1 plasmid constructs to evaluate the effects on protein stability in HEK293 cells and on the kinetics of actin repolymerisation in retinal pigment epithelium cells. Patients' sperm samples were visualised by transmission electron microscopy to determine axoneme structures and were stained with fluorescent phalloidin to visualise the fibrillar (F)-actin. A homozygous missense mutation in Ciliogenesis Associated TTC17 Interacting Protein ( This is the first report of a recessive mutation in

Sections du résumé

BACKGROUND BACKGROUND
Oligoteratoasthenozoospermia (OTA) combines deteriorated quantity, morphology and motility of the sperm, resulting in male factor infertility.
METHODS METHODS
We used whole genome genotyping and exome sequencing to identify the mutation causing OTA in four men in a consanguineous Bedouin family. We expressed the normal and mutated proteins tagged with c-Myc at the carboxy termini by transfection with pCDNA3.1 plasmid constructs to evaluate the effects on protein stability in HEK293 cells and on the kinetics of actin repolymerisation in retinal pigment epithelium cells. Patients' sperm samples were visualised by transmission electron microscopy to determine axoneme structures and were stained with fluorescent phalloidin to visualise the fibrillar (F)-actin.
RESULTS RESULTS
A homozygous missense mutation in Ciliogenesis Associated TTC17 Interacting Protein (
CONCLUSION CONCLUSIONS
This is the first report of a recessive mutation in

Identifiants

pubmed: 32503832
pii: jmedgenet-2019-106825
doi: 10.1136/jmedgenet-2019-106825
pii:
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Informations de copyright

© Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.

Déclaration de conflit d'intérêts

Competing interests: None declared.

Auteurs

Maram Arafat (M)

The Shraga Segal Department of Microbiology, Immunology & Genetics, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheva, Israel.
The National Institute for Biotechnology in the Negev, Ben-Gurion University of the Negev, Beer Sheva, Israel.

Avi Harlev (A)

Fertility and IVF Unit, Department of Obstetrics and Gynecology, Soroka University Medical Center, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheva, Israel.

Iris Har-Vardi (I)

Fertility and IVF Unit, Department of Obstetrics and Gynecology, Soroka University Medical Center, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheva, Israel.

Eliahu Levitas (E)

Fertility and IVF Unit, Department of Obstetrics and Gynecology, Soroka University Medical Center, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheva, Israel.
The Center of Advanced Research and Education in Reproduction (CARER), Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheva, Israel.

Tsvia Priel (T)

Fertility and IVF Unit, Department of Obstetrics and Gynecology, Soroka University Medical Center, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheva, Israel.

Moran Gershoni (M)

ARO- The Volcani Center, Institute of Animal Science, Rehovot - Faculty of Agriculture Bet Dagan, Rishon LeZion, Israel.

Charles Searby (C)

Department of Pediatrics and Ophthalmology, Division of Medical Genetics, University of Iowa, Iowa City, Iowa, USA.

Val C Sheffield (VC)

Department of Pediatrics and Ophthalmology, Division of Medical Genetics, University of Iowa, Iowa City, Iowa, USA.

Eitan Lunenfeld (E)

Fertility and IVF Unit, Department of Obstetrics and Gynecology, Soroka University Medical Center, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheva, Israel.
The Center of Advanced Research and Education in Reproduction (CARER), Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheva, Israel.

Ruti Parvari (R)

The Shraga Segal Department of Microbiology, Immunology & Genetics, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheva, Israel ruthi@bgu.ac.il.
The National Institute for Biotechnology in the Negev, Ben-Gurion University of the Negev, Beer Sheva, Israel.

Classifications MeSH