Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019270 (hernia)
15,856 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The mouse bone morphogenetic protein1 (Bmp1) gene encodes a secreted astacin metalloprotease that cleaves the COOH-propeptide of procollagen I, II and III. BMP-1 is also related to the product of the Drosophila patterning gene, tolloid (tld), which enhances the activity of the TGFbeta-related growth factor Decapentaplegic and promotes development of the dorsalmost amnioserosa. We have disrupted the mouse Bmp1 gene by deleting DNA sequences encoding the active site of the astacin-like protease domain common to all splice variants. Homozygous mutant embryos appear to have a normal skeleton, apart from reduced ossification of certain skull bones. However, they have a persistent herniation of the gut in the umbilical region and do not survive beyond birth. Analysis of the amnion of homozygous mutant embryos reveals the absence of the fold that normally tightly encloses the physiological hernia of the gut. At the electron microscopic level, the extracellular matrix of the amnion contains collagen fibrils with an abnormal morphology, consistent with the incorporation of partially processed procollagen molecules. Metabolical labelling and immunofluorescence studies also reveal abnormal processing and deposition of procollagen by homozygous mutant fibroblasts in culture.
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PMID:Failure of ventral body wall closure in mouse embryos lacking a procollagen C-proteinase encoded by Bmp1, a mammalian gene related to Drosophila tolloid. 895 Oct 74

The purpose of this study was to investigate different forms of the local application of TGF-beta(1) for augmentation of the anterior abdominal wall in an appropriate model of an incisional hernia. Sixty male Sprague-Dawley rats were divided into six groups. Artificial defects of the anterior abdominal wall were closed with one of the following methods: running Prolene suture, Vicryl mesh, prolene suture followed by an intramuscular injection of 1 mug TGF-beta(1), Vicryl mesh coated with 1 mug TGF-beta(1), and prolene suture coated with 1 mug TGF-beta(1). A control group did not receive any defect and treatment. Six weeks after operation, tensile strength, collagen content, gene expression of collagen I and III, blood vessels, and thickness of collagen fibres were evaluated. Tensile strength was strongest in the controls (14.2 (10.5-18 N)). There was no increase in tensile strength due to the administration of TGF-beta(1). On the contrary, bolus injection of the growth factor resulted in a significantly decreased strength of the wound tissue when compared to the groups 1, 4, 5, and 6 (9.1 (4.2-9.1 N)). These results correlated with the gene expression of collagen I and III. Local application of TGF-beta(1) did not augment the strength of the abdominal wall after 6 weeks.
Hernia 2005 Oct
PMID:Local administration of TGF-beta1 to reinforce the anterior abdominal wall in a rat model of incisional hernia. 1591 58

Transforming growth factor (TGF)-beta signalling plays important roles in regulating lung development. However, the specific regulatory functions of TGF-beta signalling in developing lung epithelial versus mesenchymal cells are still unknown. By immunostaining, the expression pattern of the TGF-beta type II receptor (TbetaRII) was first determined in the developing mouse lung. The functions of TbetaRII in developing lung were then determined by conditionally knocking out TbetaRII in the lung epithelium of floxed-TbetaRII/surfactant protein C-reverse tetracycline transactivator/TetO-Cre mice versus mesenchyme of floxed-TbetaRII/Dermo1-Cre mice. TbetaRII was expressed only in distal airway epithelium at early gestation (embryonic day (E)11.5), but in both airway epithelium and mesenchyme from mid-gestation (E14.5) to post-natal day 14. Abrogation of TbetaRII in mouse lung epithelium resulted in retardation of post-natal lung alveolarisation, with markedly decreased type I alveolar epithelial cells, while no abnormality in prenatal lung development was observed. In contrast, blockade of TbetaRII in mesoderm-derived tissues, including lung mesenchyme, resulted in mildly abnormal lung branching and reduced cell proliferation after mid-gestation, accompanied by multiple defects in other organs, including diaphragmatic hernia. The primary lung branching defect was verified in embryonic lung explant culture. The novel findings of the present study suggest that transforming growth factor-beta type II receptor-mediated transforming growth factor-beta signalling plays distinct roles in lung epithelium versus mesenchyme to differentially control specific stages of lung development.
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PMID:TGF-beta receptor II in epithelia versus mesenchyme plays distinct roles in the developing lung. 1832 28