PubMed Health⌕ Search

Biomedical subjects

Shota Narai

Publications and source records attributed to Shota Narai.

2 recordsLinked to original sources
PubMed2006

Blepharo-cheilo-dontic (BCD) syndrome: case report.

OBJECTIVE: To report a case of blepharo-cheilo-dontic (BCD) syndrome, to compare with the previous cases, and to discuss the possible treatment. PATIENT: The patient was a 14-day-old boy born uneventfully on August 7, 2002. His scalp hair was sparse and curly. A tumor on the top of his head, which was thought to be a dermoid cyst, and a nevus on the back of his left hand were observed. All typical symptoms of blepharo-cheilo-dontic syndrome, such as euryblepharon with ectropion of the lower eyelids, distichiasis, bilateral cleft lip and palate, severe oligodontia, and conical teeth, were observed. Because there was no fatal complication, and growth and development were about normal, it was recommended that a long and active treatment plan be considered for this patient.

Cleft Lip↗
PubMed2006

Trp53 affects the developmental anomaly of clefts of the palate in irradiated mouse embryos but not clefts of the lip with or without the palate.

Trp53-deficient mice exhibit increased incidences of developmental anomalies when irradiated, probably due to lack of Trp53-dependent apoptosis. A/J strain-derived CL/Fr mice develop clefts of the lip with or without the palate (CL/P) in approximately one-fifth of the embryos. We produced Trp53-deficient CL/Fr mice and examined the susceptibility to spontaneous development of CL/P and clefts of palate only (CPO), which differ in their developmental mechanisms, CL/P resulting from clefts of the primary palate and CPO from clefts of the secondary palate. The effect of radiation on the two phenotypes was also studied. Unexpectedly, no increase in the frequency of CL/P was observed under either condition, indicating that Trp53 deficiency does not contribute to genesis of CL/P. On the other hand, radiation enhanced the incidence of CPO in Trp53(+/+) embryos but not in Trp53(+/-) and Trp53(-/-/) embryos, suggesting that the absence or presence of only one allele of Trp53 is insufficient to hinder differentiation and proliferation of cells involved in the secondary palate formation. These results indicate that Trp53 function adversely affects the development of CPO when certain damaging agents such as radiation are given.

Animals↗