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Pediatr Res

Title:		Gastroschisis in mice lacking aortic carboxypeptidase-like protein is associated with a defect in neuromuscular development of the eviscerated intestine
Author(s):		Danzer E; Layne MD; Auber F; Shegu S; Kreiger P; Radu A; Volpe M; Adzick NS; Flake AW;
Address:		"The Center for Fetal Research, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA"
Journal Title:		Pediatr Res
Year:		2010
Volume:		68
Issue:		1
Page Number:		23 - 28
DOI:		10.1203/PDR.0b013e3181e17c75
ISSN/ISBN:		1530-0447 (Electronic) 0031-3998 (Linking)
Abstract:		"Mice lacking aortic carboxypeptidase-like protein (ACLP) exhibit a gastroschisis (GS) like abdominal wall defect. The objectives of this study were to evaluate the pathophysiological features of GS in ACLP mice and to characterize the neuromuscular development of the eviscerated intestine (EI). ACLP mice were created by heterozygous mating from previously generated mice with targeted disruption of ACLP. Specimens were processed for H&E, and immunohistochemistry for smooth muscle cells [SMC, alpha-smooth muscle actin (alpha-SMA) antibody], interstitial cells of Cajal (ICC, c-kit-antibody), neural crest cells (NCC, Hox-b5-antibody), and enteric neurons (EN, PGP9.5-, alpha-internexin, and synaptophysin antibody). From 47 fetuses genotyped, 13 (27.7%) were wild type, 20 (42.5%) were heterozygous, and 14 (29.8%) were ACLP homozygous. In GS mice, expression of c-kit, Hox-b5, PGP-9.5, alpha-internexin, and synaptophysin were almost completely absent and only faint alpha-SMA expression was seen in the EI. In contrast, c-kit, Hox-b5, PGP9.5, alpha-internexin, synaptophysin, and alpha-SMA expression in intra-abdominal intestine in GS fetuses was the same as control intestine. The defect observed in ACLP mice closely resembles GS. Absence of ICC, NCC, EN, and immature differentiation of SMC supports an associated defect in neuromuscular development that is restricted to the EI"
Keywords:		Animals Biomarkers/metabolism Carboxypeptidases/genetics/*metabolism Female Fetus/anatomy & histology/pathology Gastroschisis/*pathology/*physiopathology Humans Interstitial Cells of Cajal/cytology/metabolism *Intestines/abnormalities/anatomy & histology/;
Notes:		"MedlineDanzer, Enrico Layne, Matthew D Auber, Frederic Shegu, Shincy Kreiger, Portia Radu, Antoneta Volpe, Maryann Adzick, N Scott Flake, Alan W eng 2010/04/14 Pediatr Res. 2010 Jul; 68(1):23-8. doi: 10.1203/PDR.0b013e3181e17c75"