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Biallelic mutations of CFAP74 may cause human primary ciliary dyskinesia and MMAF phenotype.
- Source :
-
Journal of human genetics [J Hum Genet] 2020 Nov; Vol. 65 (11), pp. 961-969. Date of Electronic Publication: 2020 Jun 18. - Publication Year :
- 2020
-
Abstract
- Primary ciliary dyskinesia (PCD) is a rare genetic disorder characterized by recurrent respiratory infections, nasosinusitis, tympanitis, and/or male infertility, all of which can severely impair the patient's quality of life. Multiple morphological abnormalities of the sperm flagella (MMAF) is one type of severe teratozoospermia and results from a variety of flagellar defects. In this study, we conducted whole-exome sequencing to identify and evaluate the genetic lesions in two patients with potential PCD and MMAF. Biallelic mutations in exon 10, c.983G>A; p.(Gly328Asp), and exon 29, c.3532G>A; p.(Asp1178Asn), of the CFAP74 (NM_001304360) gene were identified in patient 1 (P1), and biallelic mutations in exon 7, c.652C>T; p.(Arg218Trp), and exon 35, c. 4331G>C; p.(Ser1444Thr), of the same gene were identified in patient 2 (P2). Bioinformatic analysis suggested that these variants may be disease causing. Immunofluorescence confirmed that CFAP74 was absent in these patients' sperm samples. Intracytoplasmic sperm injection (ICSI) was carried out for P1, and his wife became pregnant after embryo transfer and gave birth to a healthy baby. To the best of our knowledge, this study is the first to identify the importance of CFAP74 in potential PCD and MMAF, contributing to the genetic diagnosis of these disorders and helping to predict pregnancy outcomes relevant in in vitro fertilization.
- Subjects :
- Abnormalities, Multiple pathology
Adult
Alleles
Ciliary Motility Disorders complications
Ciliary Motility Disorders pathology
Female
Flagella genetics
Flagella pathology
Genetic Predisposition to Disease
Humans
Infertility, Male complications
Infertility, Male pathology
Male
Mutation genetics
Sperm Tail metabolism
Sperm Tail pathology
Spermatozoa abnormalities
Spermatozoa metabolism
Teratozoospermia complications
Teratozoospermia pathology
Exome Sequencing
Abnormalities, Multiple genetics
Ciliary Motility Disorders genetics
Infertility, Male genetics
Teratozoospermia genetics
Subjects
Details
- Language :
- English
- ISSN :
- 1435-232X
- Volume :
- 65
- Issue :
- 11
- Database :
- MEDLINE
- Journal :
- Journal of human genetics
- Publication Type :
- Academic Journal
- Accession number :
- 32555313
- Full Text :
- https://doi.org/10.1038/s10038-020-0790-2