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. 2014 Jul 7;5:195. doi: 10.3389/fgene.2014.00195

Table 2.

Mouse Models related to Ovarian Dysfunction and FMR1.

Mutant phenotype 130R (Hoffman et al., 2012) TG206/YAC90R (Lu et al., 2012)
Alteration(s) in follicle number Decreased numbers of primordial follicles in 7–9 month-old mice Decreased numbers of growing follicles in 25 day-old and 9 week-old mice
Alteration(s) in fecundity Not evaluated Increased age at first litter, decreased number of pups per litter
Alteration(s) in hormone production Not evaluated Hormone 8–10 weeks 15–22 weeks
Estradiol ↑* **
FSH ↑* ↑*
LH ↓ n.s. ↓ n.s.
*P < 0.05, **not evaluated
Follicle death Increased histomorphometric evidence of follicle atresia in PM ovaries; increased ubiquitylated proteins in PM oocytes. Increased numbers of TUNEL-positive apoptotic cells in PM ovaries
Alterations in follicle growth Granulosa cell number is decreased in growing follicles. Cumulus granulosa cell number is also decreased in mature follicles. mTOR pathway is downregulated in whole ovaries (McLaughlin et al., 2011; Yu et al., 2011)
Alterations in gene expression Reduced expression of FMRP in PM granulosa cells. Redistribution of FMRP to oocyte nucleus in PM (FMRP is cytoplasmic in wild-type oocytes). Reduced CX36 expression. Decreased expression of LH-induced ovulation-related genes in PM ovaries versus wild-type controls.
Other abnormalities Presence of ovarian cysts; zona pellucida abnormalities. Reduced whole body weight in PM animals versus wild-type controls.