The Role of the Cloacae Membrane in Exstrophy: The First Successful Experimental Study

Muecke, Edward C.

doi:10.1016/s0022-5347(17)64028-x

Cited by 185 publications

(58 citation statements)

References 6 publications

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“…Although advanced parental age (Boyadjiev et al, 2004), familial links (Shapiro et al, 1984) and racial predilection (Roberts et al, 1995) imply a genetic cause, the molecular mechanisms underlying the formation of BE remain unknown. As such, our understanding of the pathogenesis of BE remains limited to that provided by a previous descriptive study (Muecke, 1964).…”

Section: Introductionmentioning

confidence: 99%

ΔNp63 plays an anti-apoptotic role in ventral bladder development

et al. 2006

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The bladder, the largest smooth-muscle organ in the human body, is responsible for urine storage and micturition. P63, a homolog of the p53 tumor-suppressor gene, is essential for the development of all stratified epithelia, including the bladder urothelium. The N-terminal truncated isoform of p63, ⌬Np63, is known to have anti-apoptotic characteristics. We have established that ⌬Np63 is not only the predominant isoform expressed throughout the bladder, but is also preferentially expressed in the ventral bladder urothelium during early development. We observed a host of ventral defects in p63 -/-embryos, including the absence of the abdominal and ventral bladder walls. This number of ventral defects is identical to bladder exstrophy, a congenital anomaly exhibited in human neonates. In the absence of p63, the ventral urothelium was neither committed nor differentiated, whereas the dorsal urothelium was both committed and differentiated. Furthermore, in p63 -/-bladders, apoptosis in the ventral urothelium was significantly increased. This was accompanied by the upregulation of mitochondrial apoptotic mediators Bax and Apaf1, and concurrent upregulation of p53. Overexpression of ⌬Np63␥ and ⌬Np63␤ in p63 -/-bladder primary cell cultures resulted in a rescue, evidenced by significantly reduced expressions of Bax and Apaf1. We conclude that ⌬Np63 plays a crucial anti-apoptotic role in normal bladder development.

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Section: Introductionmentioning

confidence: 99%

ΔNp63 plays an anti-apoptotic role in ventral bladder development

et al. 2006

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“…For congenital malformations in particular, comprehension of the development and improvement of treatment are most of the time based on experimental studies in animal fetuses or embryos [1,2]. The procedure may be biochemical [3,4], surgical [5][6][7][8][9], or even magnetic [10]. Nevertheless, especially in embryos, the critical point is to choose the appropriate developmental stage to perform a study, in order to obtain the most satisfactory results [5,11].…”

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confidence: 99%

Developmental Stages in the Rabbit Embryo: Guidelines to Choose an Appropriate Experimental Model

Beaudoın

Barbet²,

Bargy

2003

Fetal Diagn Ther

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Researchers involved in the field of congenital malformations are often forced to work on an animal model. Both accurate description of its normal development and comparative staging with human development will be mandatory. To complete the lacking medical literature, we herein provide such data for the rabbit model. Sampled rabbit embryos were staged using the Carnegie criteria, in order first to determine if they were consistent with the rabbit developmental pattern, and second to compare this pattern with the human one. Our results show a suitable comparison of rabbits and humans in early developmental stages, except for the neural growth.

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“…The classical picture of bladder exstrophy is the most commonly observed and occurs in 1 of every 30,000-50,000 births [1], Marshall and Muecke [7,8] described the embryogenesis of the various types of incomplete bladder exstrophy and emphasized that the typical wide separation of the abdominal wall muscles and the pubic bones is a main feature of all exstrophic anomalies.…”

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confidence: 99%