Gene analyses and neural crest stem cell therapy in Hirschsprung's disease and allied disorders.

2014 Fiscal Year Final Research Report

• Project/Area Number: 24592700
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Pediatric surgery
• Research Institution: Keio University
• Principal Investigator: SHIMOJIMA Naoki 慶應義塾大学, 医学部, 講師 (30317151)
• Co-Investigator(Kenkyū-buntansha): KURODA Tatsuo 慶應義塾大学, 医学部, 教授 (60170130) ; SHIBATE Shinsuke 慶應義塾大学, 医学部, 講師 (70407089)
• Co-Investigator(Renkei-kenkyūsha): OKANO Hideyuki 慶應義塾大学, 医学部, 教授 (60160694) ; KUDO Jun 慶應義塾大学, 医学部, 教授 (80178003) ; KOSAKI Kenjiro 慶應義塾大学, 医学部, 教授 (30234743) ; FUJIMURA Takumi 慶應義塾大学, 医学部, 助教 (80573443)
• Project Period (FY): 2012-04-01 – 2015-03-31
• Keywords: 腸管神経 / 発生 / 分化 / 幹細胞 / 再生医療 / 神経堤細胞

Outline of Final Research Achievements

A final goal of our project is to establish a cell transplant therapy for Hirschsprung's disease and allied disorders. Understanding the etiology of diseases is very important, so we began with gene analyses using human samples from patients with hypoganglionosis. To date, we identified 2 candidate genes which might be causative genes.
For a better understanding of etiology of hypoganglionosis, populations of ganglion cells and glial cells were analyzed quantitatively. Immunohistochemical staining for enteric nerves and glial cells were performed. Ganglion cells and glial cells were both decreased in number but the ratio of glial cells to ganglion cells was significantly lower in hypoganglionosis. Thus, the result from a disturbed generation of trophic factors by accompanying glial cells might be an etiological factor for the decreased number of ganglion cells in hypoganglionosis.

Free Research Field

小児外科学