Changes in regulatory molecules for lymphangiogenesis in intestinal lymphangiectasia with enteric protein loss.
ABSTRACT Vascular endothelial growth factor receptor 3 (VEGFR3) and LYVE-1 are specifically expressed in the endothelium of the lymphatic systems. VEGF-C, D, FOXC2, Prox 1, and SOX18 are known to play central roles in lymphatic development. We investigated the expression of regulatory molecules for lymphangiogenesis in the duodenal mucosa of idiopathic intestinal lymphangiectasia.
Biopsy samples were obtained from duodenal biopsies in patients with intestinal lymphangiectasia complicated with protein-losing from white spot lesions in which lymphangiectasia was histologically confirmed. Immunohistochemical analysis for VEGFR3 and LYVE-1 was performed. mRNA expression of VEGF-C, VEGF-D, VEGFR3, and transcription factors was determined by the quantitative reverse transcription-polymerase chain reaction method.
In the control mucosa, VEGFR3 was weakly expressed on the central lymphatic vessels in the lamina propria and LYVE-1 was expressed mainly on the lymphatic vessels in the submucosa. In intestinal lymphangiectasia, VEGFR3 and LYVE-1 expression levels were increased on the mucosal surface corresponding to widely dilated lymphatic vessels, while they were decreased in the deeper mucosa. mRNA expression study showed a significant increase in the expression level of VEGFR3 in lymphangiectasia, but the expression of VEGF-C and -D mRNA was significantly suppressed compared with that in controls despite the presence of lymphangiectasia. The mRNA expression levels of FOXC2 and SOX18 were also decreased, whereas Prox 1 was not altered.
There is an altered expression of regulatory molecules for lymphangiogenesis in the duodenal mucosa in these patients.
- SourceAvailable from: europepmc.org[Show abstract] [Hide abstract]
ABSTRACT: Intestine has a well-developed lymphatic system that is closely related with its functions, such as mucosal immunological defense or absorption of nutrients. Intestinal lymphoid cells such as lymphocytes, macrophages/monocytes, or dendritic cells are continuously migrating through intestinal mucosa, thereby facilitating their immune responses. Their migrations are well controlled by well-organized molecular mechanisms including adhesion molecules, chemokines, etc. This manuscript will review how dysfunction of lymphoid cell migration is involved in intestinal inflammation, especially in the pathophysiology of intestinal bowel diseases. (*English Translation of J Jpn Coll Angiol 2008; 48: 143-149.).Annals of Vascular Diseases 01/2012; 5(3):275-81.
- [Show abstract] [Hide abstract]
ABSTRACT: Our understanding of the genes and mechanisms controlling the formation of the lymphatic vasculature during embryonic development has improved a great deal during the last decade. The availability of molecular markers that allow us to distinguish the lymphatic vasculature from the blood vasculature and the generation of mouse models with various degrees of lymphatic defects have been instrumental to the progress in this field. In this chapter, we highlight some of the key molecular players that regulate the development of the lymphatic vasculature and some available mouse models of lymphatic disorders.09/2009: pages 11-25;
- [Show abstract] [Hide abstract]
ABSTRACT: Primary idiopathic intestinal lymphangiectasia is an unusual disease featured by the presence of dilated lymphatic channels which are located in the mucosa, submucosa or subserosa leading to protein loosing enteropathy.Most often affected were children and generally diagnosed before third year of life but may be rarely seen in adults too. Bilateral pitting oedema of lower limb is the main clinical manifestation mimicking the systemic disease and posing a real diagnostic dilemma to the clinicians to differentiate it from other common systemic diseases like Congestive cardiac failure, Nephrotic Syndrome, Protein Energy Malnutrition, etc. Diagnosis can be made on capsule endoscopy which can localise the lesion but unable to take biopsy samples. Thus, recently double-balloon enteroscopy and biopsy in combination can be used as an effective diagnostic tool to hit the correct diagnosis. Patients respond dramatically to diet constituting low long chain triglycerides and high protein content with supplements of medium chain triglyceride. So early diagnosis is important to prevent untoward complications related to disease or treatment for the sake of accurate pathological diagnosis.World journal of clinical cases. 10/2014; 2(10):528-33.