Introduction 1993 1996 1997 1998 2006 1997 2001 2003 2004 2005 1998 2000 2000 1999 1999 2006 2002 2005 1998 2005 2001 2005 2001 2006a b 2006 Materials and methods TNX-deficient patients We investigated pregnancies and GU abnormalities in all currently identified female tenascin-X deficient patients of reproductive age identified in our clinic and in the literature. The study protocol was approved by the local medical ethics committee, and written informed consent was obtained from the patients. Experimental animals 2002 Breeding Breeding pairs were kept in a 5:1 female:male ratio. Females were inspected daily for vaginal plugs, and those with plugs were isolated and inspected each day for abnormalities and progression of pregnancy. Litter size was determined after birth. Neonatal survival is defined as the difference in litter size after partus and 2 weeks postpartum. Processing of samples for (immuno)histochemistry 2 Affinity-purified TNX antibody production 1989 Eco Sal Eco Sal Histopathology, histochemistry, and immunohistochemistry 2005 2006b 2006 2007 2006b Electron microscopy 1993 Statistics P Results Pregnancy in TNX-deficient patients 1 2003 1 2005 1993 2000 2005 2002 2006 Table 1 VUE IUD CAH Patient ID Age (years) Gravida number/Para number Genito-urinary complications Specification 1 50 G1P1 None None 2 (sister of patient 1) 60 G2P2  VUE after uterine prolapse (at age 49) None  Vaginal prolapse 2 weeks after VUE None 3 46 G0P0 None Was born prematurely (week 35) with shoulder luxation, rectal prolapse at age 1  4 (sister of patient 3) 51 G3P3  One incident of hemorrhage postpartum (>1000 ml blood loss) None 5 38 G0P0 None CAH  6 51 G3P2  IUD (24 weeks) with relatively large blood loss 2001  Undefined prolapse (at age 36) 7 57* G4P4  Precipitious second stage at term for all births 2005  Uterine prolapse (at age 20), also recurrent rectal prolapses Pregnancy in TNX KO mice t 2 1 1 Table 2 Pregnancy in TNX-deficient mice Genito-urinary complications TNX KO mice WT mice Number of mice Number of mice Maternal death during pregnancy 1 0 Miscarriage 2 0 Obstruction during partus 1 3 Excessive bleeding of vagina (as seen at vaginal plug check) 2 0 Cannibalism after partus 1 1 Total number of evaluated pregnancies 36 31 Fig. 1 a P n n b  Structure of mouse uterus 2 Fig. 2 a b P M E L Bars  TNX distribution in mouse uterus 3 3 3 3 3 Fig. 3 P M M* E Epi L green a b c blue d f a-c f d e g d–g Bars  Collagen distribution in mouse uterus 2006a b 4 2006b 2006 5 5 5 Fig. 4 P M M* E Epi L b e h green a d g red c f i yellow orange magenta k l m Bars  Fig. 5 P M E red M* BV L a c e g b d f h Bars  Elastic fibers in mouse tissues 2004 6 6 6 6 6 6 6 2006a b 6 2006a b 2006b 2004 7 7 7 7 2004 7 7 Fig. 6 L purple M M* P E a b red M* c d M P red E c d red orange green M* e 4 Bars  Fig. 7 a b arrows arrows c d e arrow f g h arrows Bars a b c d e g h f  Discussion 2000 2006 2002 2005 1998 2000 2006 2005 1998 2000 2002 2000 2002 1998 Complete TNX deficiency in humans is a rare condition, and so far, only a few patients have been identified. We have therefore investigated pregnancy and uterine tissue structure in our TNX KO mouse model. No gross significant differences have been found with regard to abnormalities during pregnancy or reproduction between the TNX KO and WT mice. A trend toward a reduction in the length of pregnancy in TNX KO mice. The only significant difference has been observed in the location of the vaginal plugs, which suggests laxity in the vaginal wall. 2006b 2001 2004 2006b 2002 2004 2006b 2005 1997 2006b 2005 2002 2001 2004 2001 2004