Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Institute of Reproductive and Stem Cell Engineering, |
RCV000856592 | SCV001190586 | pathogenic | Spermatogenic failure 40 | 2019-08-14 | criteria provided, single submitter | research | WES was performed to analyse the genetic etiology of a cohort of 47 individuals with severe asthenozoospermia from 45 unrelated Chinese families. One homozygous non-sense mutation (NM_194302, c.G5341T:p.E1781X), two compound heterozygous mutations (c.C2284T:p.R762X and c.1751delC:p.P584fs) and two compound heterozygous mutations (c.5714_5721del:p.L1905fs and c.C3021A:p.N1007K) were identified in CFAP65 of three individuals with completely immotile spermatozoa, respectively. No biallelic deleterious variants of CFAP65 were detected in the control cohort of 637 individuals. Ultrastructural and immunostaining analyses of spermatozoa from two patients showed highly aberrant sperm morphology with severe defects such as acrosome hypoplasia, disruption of the mitochondrial sheath and absence of the central pair complex. |
OMIM | RCV000856592 | SCV000999090 | pathogenic | Spermatogenic failure 40 | 2020-07-21 | no assertion criteria provided | literature only |