Germ cell migration and early development of the gonads in the trisomy 16 mouse — an animal model for Down's syndrome

Leffler, Andreas; Ludwig, Michael; Schmitt, Oliver; Busch, Lüder C.

doi:10.1016/s0940-9602(99)80039-9

Cited by 11 publications

(8 citation statements)

References 30 publications

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“…The decreased bone turnover is unexpected, given the hypogonadism and infertility associated with the disorder in humans [28] and in Ts65Dn mice [29]. In fact, numerous published studies have yet to completely elucidate the pathophysiology of the infertility in men with Down syndrome [28], [30], [31], [32].…”

Section: Discussionmentioning

confidence: 99%

Low Bone Turnover and Low BMD in Down Syndrome: Effect of Intermittent PTH Treatment

et al. 2012

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Trisomy 21 affects virtually every organ system and results in the complex clinical presentation of Down syndrome (DS). Patterns of differences are now being recognized as patients’ age and these patterns bring about new opportunities for disease prevention and treatment. Low bone mineral density (BMD) has been reported in many studies of males and females with DS yet the specific effects of trisomy 21 on the skeleton remain poorly defined. Therefore we determined the bone phenotype and measured bone turnover markers in the murine DS model Ts65Dn. Male Ts65Dn DS mice are infertile and display a profound low bone mass phenotype that deteriorates with age. The low bone mass was correlated with significantly decreased osteoblast and osteoclast development, decreased bone biochemical markers, a diminished bone formation rate and reduced mechanical strength. The low bone mass observed in 3 month old Ts65Dn mice was significantly increased after 4 weeks of intermittent PTH treatment. These studies provide novel insight into the cause of the profound bone fragility in DS and identify PTH as a potential anabolic agent in the adult low bone mass DS population.

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Section: Discussionmentioning

confidence: 99%

Low Bone Turnover and Low BMD in Down Syndrome: Effect of Intermittent PTH Treatment

et al. 2012

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“…Interestingly, targeted mutation of Francc in mice results in signi fi cantly slower proliferation of PGC [ 131 ] , suggesting again shared control mechanisms of PGC proliferation in these species. Finally, in Trisomy 16 of mouse, an animal model of the human Down's syndrome leading frequently to sub or infertility, a delay in migration and reduction of PGC number was observed [ 132 ] . Gene expression studies on single human PGC obtained from 10-week-old embryos have been reported [ 133,134 ] .…”

Section: Proliferation Of Human Pgcsmentioning

confidence: 99%

Origin, Migration, and Proliferation of Human Primordial Germ Cells

Felici

2012

Oogenesis

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The fi rst histological observations about the origin of the precursors of gametes termed primordial germ cells (PGCs) in extragonadal regions and their subsequent migration into the developing gonads in human embryos date back to the early twentieth century. Fuss (Anat Am 39:407-409, 1911, Anat EntwMech 81:1-23, 1912 and Felix (Die Entwicklung der Harn-und Geschlechtsorgane. In: Keibel-Mall Handbuch der 1qEntwick-lungageschichte des Menshen, vol 2. Leipzig, Hirzel, pp 732-955, 1911) were apparently the fi rst ones to describe the extragonadal location of PGCs in human embryos. In the youngest, 2.5 mm long, embryo examined (23-26 days postfertilization), These authors described PGCs in the endoderm of the yolk sac wall as cells identi fi able by their large size and spherical shape. Subsequently, Politzer (Z Anat Entw Gesch 87: 766-80, 1928, Z Anat Entw Gesch 93:386-428, 1930, Z Anat EntwGesch 100:331-336, 1933 and Witschi (Contr Embryol Carnegie Inst 209:67-80, 1948) studied the distribution of PGCs in a considerable number of embryos from presomite stages (0.3-0.8 mm, about 3 weeks) to 8.5 mm (5 weeks). Both authors described the migration of PGCs from the yolk sac to the developing gonads. Following a hot debate, it is now generally accepted that after their arrival into the gonadal anlage, PGCs give rise to the oogonia/oocytes and gonocytes (or prespermatogonia) in the embryonic ovary and testis, respectively. These germ cells enter a complex series of events that in the

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“…These excessive accumulation of basal lamina material and muscularis mucosa interfered with normal migration of precursor cells [47][48][49][50][51] . Payette and associates [52] thought that the excessive accumulation of components of basal lamina material in the aganglionic region of the lethal spotted mutant mouse stopped precursor cells from migrating into the terminal bowel of lethal spotted mutant mice.…”

Section: Discussionmentioning

confidence: 99%

The development of colon innervation in trisomy 16 mice and Hirschsprungs disease

Mi²,

Zhou³

et al. 2001

WJG

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AIM To study the colon innervation of trisomy 16 mouse, an animal model for Down's syndrome, and the expression of protein gene product 9.5 (PGP 9.5) in the stenosed segment of colon in Hirschsprungs disease (HD). METHODS Trisomy 16 mouse breeding; cytogenetic analysis of trisomy 16 mice;and PG P9.5 immunohistochemistry of colons of trisomy 16 mice and HD were carried out. RESULTS Compared with their normal littermates, the nervous system of colon in trisomy 16 mice was abnormally developed. There existed developmental delay of muscular plexuses of colon, no submucosal plexus was found in the colon, and there was 5 mm aganglionic bowel aparting from the anus in trisomy 16 mice. The mesentery nerve fibers were as well developed as shown in their normal littermates. Abundant proliferation of PGP 9.5 positive nerve fibers was evealed in the stenosed segment of HD colon. CONCLUSION Trisomy 16 mice could serve as an animal model for Hirschsprung's disease for aganglionic bowel in the distal part of colon. Abundant proliferation of PGP 9.5 positive fibers resulted from extrinsic nerve compensation, since no ganglionic cells were observed in the stenosed segment of the colon in HD. HD has a genetic tendency.

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Germ cell migration and early development of the gonads in the trisomy 16 mouse — an animal model for Down's syndrome

Cited by 11 publications

References 30 publications

Low Bone Turnover and Low BMD in Down Syndrome: Effect of Intermittent PTH Treatment

Low Bone Turnover and Low BMD in Down Syndrome: Effect of Intermittent PTH Treatment

Origin, Migration, and Proliferation of Human Primordial Germ Cells

The development of colon innervation in trisomy 16 mice and Hirschsprungs disease

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