nach oben

Pediatric Surgery International

Erschienen in:

23.09.2016 | Original Article

Altered differentiation of enteric neural crest-derived cells from endothelin receptor-B null mouse model of Hirschsprung’s disease

verfasst von: Naho Fujiwara, Katsumi Miyahara, Nana Nakazawa-Tanaka, Chihiro Akazawa, Atsuyuki Yamataka

Erschienen in: Pediatric Surgery International | Ausgabe 12/2016

Einloggen, um Zugang zu erhalten

Abstract

Purpose

Hirschsprung’s disease (HD) is caused by a failure of enteric neural crest-derived cells (ENCC) to colonize the bowel, resulting in an absence of the enteric nervous system (ENS). Previously, we developed a Sox10 transgenic version of the Endothelin receptor-B (Ednrb) mouse to visualize ENCC with the green fluorescent protein, Venus. The aim of this study was to isolate Sox10-Venus⁺ cells, which are differentiated neurons and glial cells in the ENS, and analyze these cells using Sox10-Venus mice gut.

Methods

The mid-and hindgut of Sox10-Venus⁺/Ednrb ^+/+ and Sox10-Venus⁺/Ednrb ^−/− at E13.5 and E15.5 were dissected and cells were dissociated. Sox10-Venus⁺ cells were then isolated. Expression of PGP9.5 and GFAP were evaluated neurospheres using laser scanning microscopy.

Results

7 days after incubation, Sox10-Venus⁺ cells colonized the neurosphere. There were no significant differences in PGP9.5 expressions on E13.5 and E15.5. GFAP was significantly increased in HD compared to controls on E15.5 (P < 0.05).

Conclusions

Our results suggest increased glial differentiation causes an imbalance in ENCC lineages, leading to a disruption of normal ENS development in this HD model. Isolation of ENCC provides an opportunity to investigate the ENS with purity and might be a useful tool for modeling cell therapy approaches to HD.

Goldstein AM, Hofstra RMW, Burns AJ (2013) Building a brain in the gut: development of the enteric nervous system. Clin Genet 83:307–316CrossRefPubMed

Xie D, Croaker GDH, Li JM, Song ZM (2016) Reduced cell proliferation and increased apoptosis in the hippocampal formation in a rat model of Hirschsprung’s disease. Brain Res 1642:79–86CrossRefPubMed

Musser MA, Southard-Smith EM (2013) Balancing on the crest—evidence for disruption of the enteric ganglia via inappropriate lineage segregation and consequences for gastrointestinal function. Dev Biol 382:356–364CrossRefPubMedPubMedCentral

Wegner M (1999) From head to toes: the multiple facets of Sox proteins. Nucl. Acids Res 27(6):1409–1420CrossRefPubMedPubMedCentral

Kawaguchi J, Nichols J, Gierl MS et al (2010) Isolation and propagation of enteric neural crest progenitor cells from mouse embryonic stem cells and embryos. Development 137:693–704CrossRefPubMedPubMedCentral

Hearn CJ, Murphy M, Newgreen D (1998) GDNF and ET-3 differentially modulate the numbers of avian enteric neural crest cells and enteric neurons in vitro. Dev Biol 197:93–105CrossRefPubMed

Nagy N, Goldstein AM (2006) Endothelin-3 regulated neural crest cell proliferation and differentiation in the hindgut enteric nervous system. Dev Biol 293:203–217CrossRefPubMed

Wu JJ, Chen JX, Rothman TP et al (1999) Inhibition of in vitro enteric neuronal development by endothelin-3: mediation by endothelin B receptors. Development 126:1161–1173PubMed

Barlow A, de Graaff E, Pachnis V (2003) Enteric nervous system progenitors are coordinately controlled by the G protein-coupled receptor EDNRB and the receptor tyrosine kinase RET. Neuron 40:905–916CrossRefPubMed

10.

Miyahara K, Kato Y, Koga H et al (2010) Abnormal enteric innervation identified without histopathologic staining in aganglionic colorectum from a mouse model of Hirschsprung’s disease. J Pediatr Surg 45:2403–2407CrossRefPubMed

11.

Shibata S, Yasuda A, Renault-Mihara F et al (2010) Sox10-Venus mice: a new tool for real-time labeling of neural crest lineage cells and oligodendrocytes. Mol Brain 3:31CrossRefPubMedPubMedCentral

12.

Bondurand N, Natarajan D, Thapar N et al (2003) Neuron and glia generating progenitors of the mammalian enteric nervous system isolated from fortal and postnatal gut cultures. Developement 130:6387–6400

13.

Grundmann D, Klotz M, Rabe H et al (2015) Isolation of high-purity myenteic plexus from adult human and mouse gastrointestinal tract. Sci Rep 20(5):9226CrossRef

14.

Xu Q, Heanue T, Pachnis V (2014) Travelling within the fetal gut: simple rules for an arduous journey. BMC Bio 30(12):50CrossRef

15.

Amiel J, Sproat-Emison E, Garcia-Barcelo M et al (2008) Hirschsprung disease, associated syndromes and genetics: a review. J Med Genet 45(1):1–14CrossRefPubMed

16.

Lake JL, Heuckroth RO (2013) Enteric nervous system development: migration, differentiation, and disease. Am J Physiol Gastrointest Liver Physiol 305(1):G1–24CrossRefPubMedPubMedCentral

17.

Young HM, Bergner AJ, Muller T (1993) Acquisition of neuronal and glial markers by neural crest-derived cells in the mouse intestine. J Comp Neurol 456(1):1–11CrossRef

18.

Heanue TA, Pachnis V (2007) Enteric nervous system development and Hirschsprung’s disease: advances in genetic and stem cell studies. Nat Rev Neurosci 8(6):466–479CrossRefPubMed

Titel: Altered differentiation of enteric neural crest-derived cells from endothelin receptor-B null mouse model of Hirschsprung’s disease
verfasst von: Naho Fujiwara
Katsumi Miyahara
Nana Nakazawa-Tanaka
Chihiro Akazawa
Atsuyuki Yamataka
Publikationsdatum: 23.09.2016
Verlag: Springer Berlin Heidelberg
Erschienen in: Pediatric Surgery International / Ausgabe 12/2016
Print ISSN: 0179-0358
Elektronische ISSN: 1437-9813
DOI: https://doi.org/10.1007/s00383-016-3964-4

Neu im Fachgebiet Pädiatrie

16.04.2024 | Stillen, Säuglingsnahrung, Beikost | Nachrichten

Update Pädiatrie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.

Newsletter bestellen

Springer Medizin

Altered differentiation of enteric neural crest-derived cells from endothelin receptor-B null mouse model of Hirschsprung’s disease

Abstract

Purpose

Methods

Results

Conclusions

Neu im Fachgebiet Pädiatrie

Stillende Frauen haben geringeres kardiovaskuläres Risiko

Komplexes Krankheitsbild zwischen Haut und Lunge – und das mit 13 Jahren

Wie toxische Männlichkeit der Gesundheit von Männern schadet

Angeborene Herzfehler: Viele sind später körperlich oder kognitiv eingeschränkt

Update Pädiatrie

Springer Medizin

Abstract

Purpose

Methods

Results

Conclusions

Bitte loggen Sie sich ein, um Zugang zu diesem Inhalt zu erhalten

Weitere Artikel der Ausgabe 12/2016

Accelerated intestinal epithelial cell turnover after bowel resection in a rat is correlated with inhibited hedgehog signaling cascade

Classification of biliary atresia in the laparoscopic era

Successful grafting of tissue-engineered fetal skin

Open versus laparoscopic approach for intestinal malrotation in infants and children: a systematic review and meta-analysis

Osmolality of enteral formula and severity of experimental necrotizing enterocolitis

Formula feeding and systemic hypoxia synergistically induce intestinal hypoxia in experimental necrotizing enterocolitis

Neu im Fachgebiet Pädiatrie

Stillende Frauen haben geringeres kardiovaskuläres Risiko

Komplexes Krankheitsbild zwischen Haut und Lunge – und das mit 13 Jahren

Wie toxische Männlichkeit der Gesundheit von Männern schadet

Angeborene Herzfehler: Viele sind später körperlich oder kognitiv eingeschränkt

Update Pädiatrie