{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T22:25:48Z","timestamp":1787005548270,"version":"build-2736575974"},"reference-count":0,"publisher":"CSIRO Publishing","issue":"7","license":[{"start":{"date-parts":[[2025,5,27]],"date-time":"2025-05-27T00:00:00Z","timestamp":1748304000000},"content-version":"vor","delay-in-days":8218,"URL":"https:\/\/doi.org\/10.1071\/journalslicense"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2002,11,26]]},"abstract":"<jats:p>Sandhoff disease is a human lysosomal storage disease. In a knockout mouse model of Sandhoff disease, which lacks the \u00df-subunit of \u00df-hexosaminidase A (Hex A, a\u00df subunits) and B (Hex B, \u00df\u00df subunits), the mutant homozygous mice ( Hexb \u2013\/\u2013) are healthy until 15 weeks of age when they develop neurodegenerative symptoms. This study was designed to analyse the fertility profile of male and female Hexb \u2013\/\u2013 mice. Mating behaviour of Hexb \u2013\/\u2013 mice was assessed at different ages. The ovarian function of Hexb \u2013\/\u2013 females was determined by superovulation studies. The quality of spermatozoa and ova was assessed by an in vitro fertilization (IVF) procedure. Hexb \u2013\/\u2013 mice were fertile at a young age. Males were fertile up to the age of 69.3 \u00b1 6.3 days (mean \u00b1&amp;nbsp;SD) and females were fertile up to the age of 56\u201363 days. Since both the Hexb \u2013\/\u2013 sexes showed fertility, the results indicate that Hex A and Hex B (major isozymes of \u00df-hexosaminidase) may not be required for sperm\u2013ovum interactions, in contrast to the widely accepted belief. On the other hand, young adult Hexb \u2013\/\u2013 males showed a reduction in mating behaviour at the age of 84.8 \u00b1 2.2 days and an absence of mating behaviour at 94.2 \u00b1 2.0 days. Spermatozoa from Hexb \u2013\/\u2013 mice (aged 109.2 \u00b1 1.8 days) showed a lower IVF rate. Among Hexb \u2013\/\u2013 females aged 85.6 \u00b1 2.1 days, no mice becamepregnant although they were positive for a vaginal plug when caged with fertile males. The number of ova recovered from Hexb \u2013\/\u2013 females (aged 111.0 \u00b1 3.1 days) and the IVF rate of ova were lower than those of controls. In conclusion, Hex A and Hex B may not be required for sperm\u2013ovum interactions. Mice lacking Hex A and Hex B activities develop infertility at a young adult age in an age-dependent manner.<\/jats:p>","DOI":"10.1071\/rd02060","type":"journal-article","created":{"date-parts":[[2003,3,6]],"date-time":"2003-03-06T17:40:32Z","timestamp":1046972432000},"page":"407-412","source":"Crossref","is-referenced-by-count":11,"title":["Development of infertility at young adult age in a mouse model of human Sandhoff disease"],"prefix":"10.1071","volume":"14","author":[{"given":"Subhash C.","family":"Juneja","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"67","container-title":["Reproduction, Fertility and Development"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/connectsci.au\/rd\/article-pdf\/14\/7\/407\/297311\/rd02060.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/connectsci.au\/rd\/article-pdf\/14\/7\/407\/297311\/rd02060.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,28]],"date-time":"2025-10-28T21:55:38Z","timestamp":1761688538000},"score":1,"resource":{"primary":{"URL":"https:\/\/connectsci.au\/rd\/article\/14\/7\/407\/47707\/Development-of-infertility-at-young-adult-age-in-a"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2002,11,26]]},"references-count":0,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2002,11,26]]}},"URL":"https:\/\/doi.org\/10.1071\/rd02060","relation":{},"ISSN":["1031-3613","1448-5990"],"issn-type":[{"value":"1031-3613","type":"print"},{"value":"1448-5990","type":"electronic"}],"subject":[],"published":{"date-parts":[[2002,11,26]]}}}