NR ABEH
AU Behrens,A.; Genoud,N.; Naumann,H.; Rülicke,T.; Janett,F.; Heppner,F.L.; Ledermann,B.; Aguzzi,A.
TI Absence of the prion protein homologue Doppel causes male sterility
QU EMBO Journal 2002 Jul 15; 21(14): 3652-8
IA http://www.pubmedcentral.gov/articlerender.fcgi?tool=pubmed&pubmedid=12110578
PT journal article
AB The agent that causes prion diseases is thought to be identical with PrPsc, a conformer of the normal prion protein PrPc. PrPc-deficient mice do not exhibit major pathologies, perhaps because they express a protein termed Dpl, which shares significant biochemical and structural homology with PrPc. To investigate the physiological function of Dpl, we generated mice harbouring a homozygous disruption of the Prnd gene that encodes Dpl. Dpl deficiency did not interfere with embryonic and postnatal development, but resulted in male sterility. Dpl protein was expressed at late stages of spermiogenesis, and spermatids of Dpl mutants were reduced in numbers, immobile, malformed and unable to fertilize oocytes in vitro. Mechanical dissection of the zona pellucida partially restored in vitro fertilization. We conclude that Dpl regulates male fertility by controlling several aspects of male gametogenesis and sperm-egg interaction.
MH Animals; Cell Separation; Flow Cytometry; Homozygote; In Situ Nick-End Labeling; Infertility, Male/*genetics; Male; Mice; Mice, Knockout; Prions/genetics/*physiology; Research Support, Non-U.S. Gov't; Reverse Transcriptase Polymerase Chain Reaction; Spermatogenesis/genetics; Testis/growth & development
AD Institute of Neuropathology, UniversitätsSpital Zürich, Schmelzbergstrasse 12, CH-8091 Zürich, Switzerland.
SP englisch
PO England