Neural Crest Cell Contribution to the Developing Circulatory System

Bergwerff, Maarten; Verberne, Marlies E.; DeRuiter, Marco C.; Poelmann, Robert E.; Gittenberger-de-Groot, Adriana C.

doi:10.1161/01.res.82.2.221

Cited by 281 publications

(209 citation statements)

References 47 publications

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“…3 F-H). Because SMCs, but not endothelial cells, of forebrain vessels are derived from NCCs (6,19), these results suggest that Brg1-null SMCs failed to mature to form a strong vessel wall. Such immature vascular wall may be fragile, leading to severe bleeding and embryonic lethality.…”

Section: Resultsmentioning

confidence: 83%

Brg1 governs distinct pathways to direct multiple aspects of mammalian neural crest cell development

Xiong²,

Shang³

et al. 2013

Proc. Natl. Acad. Sci. U.S.A.

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Development of the cerebral vessels, pharyngeal arch arteries (PAAs). and cardiac outflow tract (OFT) requires multipotent neural crest cells (NCCs) that migrate from the neural tube to target tissue destinations. Little is known about how mammalian NCC development is orchestrated by gene programming at the chromatin level, however. Here we show that Brahma-related gene 1 (Brg1), an ATPase subunit of the Brg1/Brahma-associated factor (BAF) chromatin-remodeling complex, is required in NCCs to direct cardiovascular development. Mouse embryos lacking Brg1 in NCCs display immature cerebral vessels, aberrant PAA patterning, and shortened OFT. Brg1 suppresses an apoptosis factor, Apoptosis signal-regulating kinase 1 (Ask1), and a cell cycle inhibitor, p21 cip1 , to inhibit apoptosis and promote proliferation of NCCs, thereby maintaining a multipotent cell reservoir at the neural crest. Brg1 also supports Myosin heavy chain 11 (Myh11) expression to allow NCCs to develop into mature vascular smooth muscle cells of cerebral vessels. Within NCCs, Brg1 partners with chromatin remodeler Chromodomain-helicase-DNAbinding protein 7 (Chd7) on the PlexinA2 promoter to activate PlexinA2, which encodes a receptor for semaphorin to guide NCCs into the OFT. Our findings reveal an important role for Brg1 and its downstream pathways in the survival, differentiation, and migration of the multipotent NCCs critical for mammalian cardiovascular development.N eural crest cells (NCCs) originate from the neural crest of the dorsal neural tube and migrate to many regions of the embryo, where they differentiate into a variety of local cells, including cardiovascular tissues (1). NCCs that emigrate from the neural crest of rhombomere 6-8 to pharyngeal arches and the heart are essential for the patterning of pharyngeal arch arteries (PAAs) and the cardiac outflow tract (OFT) (2, 3). These NCCs also differentiate into vascular smooth muscle cells (SMCs) of PAAs and the muscular septum of the aorta and pulmonary trunk (4, 5). In contrast, NCCs from the cephalic neural tube migrate to the face and forebrain to form craniofacial bones, as well as SMCs of facial and forebrain vessels (6). Thus, NCCs are critical for the formation of cardiac OFT and vascular supplies of large areas of the body.Disruption of NCC development, either directly or indirectly, results in many forms of human birth defects with cardiovascular malformations, including Alagille, Carpenter, Ivemark, Leopard, Williams, DiGeorge, and CHARGE syndromes (7). These syndromes involve defects in PAAs or cardiac OFT, such as coarctation of the aorta, interrupted aortic arch, pulmonary artery stenosis, double-outlet right ventricle, tetralogy of Fallot, or persistent truncus arteriosus. During PAA and OFT development, NCCs are regulated by numerous transcription factors, including Pax3, Pbx1/2/3, Tbx1/2/3/20, Msx1/2, Hand2, AP2a, Cited2, Pitx2, Sox4, Foxc1/c2/d3/h1, Fog2, Gata3/4/6, and Notch/NICD (8). Such extensive involvement of transcription factors indicates the importance of gene pr...

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Section: Resultsmentioning

confidence: 83%

Brg1 governs distinct pathways to direct multiple aspects of mammalian neural crest cell development

Xiong²,

Shang³

et al. 2013

Proc. Natl. Acad. Sci. U.S.A.

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“…Abnormalities of the branchial or aortic arches are associated with neural crest-cell aberrations and therefore may be associated with the CATCH 22 syndrome. 7,[9][10][11][12][13][14] Precursors of the trunco-aortic sac are mainly of mesodermal origin, 16 therefore AOPA is unlikely to be associated with the trunco-aortic sac abnormalities. 1 The origin of AOPA anatomically is usually very proximal, arising from the ascending aorta just distal to the aortic sinus.…”

Section: Discussionmentioning

confidence: 99%

Anomalous origin of the left pulmonary artery from the ascending aorta in two children with pulmonary atresia, subaortic ventricular septal defect and right-sided major aorto-pulmonary collateral arteries

Pepeta

Takawira

Cilliers

et al. 2011

CardioVascular Journal of Africa

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Anomalous origin of the left pulmonary artery from the ascending aorta in two children with pulmonary atresia, sub-aortic ventricular septal defect and right-sided major aorto-pulmonary collateral arteries L PEPETA, FF TAKAWIRA, PE ADAMS, NH NTSINJANA, BJ MITCHELL, AM CILLIERS AbstractWe report two rare cases of an anomalous origin of the left pulmonary artery (AOLPA) from the ascending aorta, associated with pulmonary atresia, a ventricular septal defect and a left aortic arch. The cases are unusual because AOLPA is more commonly associated with a right aortic arch and it is more usual for the right pulmonary artery to originate anomalously from the ascending aorta. The pulmonary blood supply to the right lung in both patients was absent and provided instead by major aorto-pulmonary collateral arteries which were stenosed at multiple levels. The AOLPA in both patients originated from the postero-lateral aspect of the ascending aorta just distal to the sino-tubular junction. Only one patient showed the more common association of an unusual aortic arch branching pattern in the form of an anomalous right subclavian artery.Neither patient was in heart failure and the chest X-ray in both revealed differential pulmonary perfusion with prominent vascularity of the left lung. Cardiac catheterisation showed systemic pressures within the anomalous left pulmonary artery. Karyotyping revealed normal chromosomes, and fluorescent in-situ hybridisation done in one patient was negative for chromosome 22q11.2 microdeletion. Both patients have been managed conservatively.Keywords: pulmonary atresia with ventricular septal defect, anomalous origin of pulmonary artery, collateral arteries, pulmonary hypertension, branchial arches, CATCH22 syndrome An anomalous origin of the pulmonary artery from the aorta (AOPA) is very rare, with the first case of this nature reported by Fraentzel in 1868. 1 The incidence of anomalous left pulmonary artery from the aorta (AOLPA) was reported in one study to be around 17%, 1 with anomalous origin of the right pulmonary artery (AORPA) from the ascending aorta as the more common variant of AOPA, seen in about 83% of cases. Without surgery, the mortality in patients with AOPA is extremely high, reaching as high as 80%. [2][3][4][5] This is due to severe, rapidly progressive pulmonary vascular disease and congestive cardiac failure. Both isolated AOLPA and that associated with other defects have been well described. 1,6,7 The commonest associated congenital cardiac lesion is tetralogy of Fallot, which is seen in about 75% of cases. 1 AOLPA is also associated with a right arch of the aorta in 63% of cases, and in about 38% of cases, an anomalous origin of the right subclavian artery from the descending aorta has been reported as well. 1 Of note, both our cases had a left aortic arch and had no main and right pulmonary arteries, with the only source of blood supply to the right lung being partially stenosed major aorto-pulmonary collateral arteries (MAPCAs).To the best of our knowledge, this series repr...

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“…The first suggests that there is a common inherited pathogenic factor responsible for the 2 lesions, 32,33 and the second implicates inadequately controlled hypertension as the main cause. 8 Hypertension is a known risk factor for the development of IAs, 34,35 multiple IAs, and their rupture.…”

Section: Ias and Hypertensionmentioning

confidence: 99%

“…1 Furthermore, surges in blood pressure can further increase the growth of IAs. 33 In heritable conditions in which IAs are common such as adult polycystic kidney disease, hypertension is also a highly relevant risk factor. [34][35][36] In our study, there was no relationship between a single clinic blood pressure recording and aneurysms, though a diagnosis of hypertension, as documented in the notes, appeared to confer a risk.…”

Section: Ias and Hypertensionmentioning

confidence: 99%

Results of Screening for Intracranial Aneurysms in Patients with Coarctation of the Aorta

Curtis

Bradley

Wilde

et al. 2012

AJNR Am J Neuroradiol

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Neural Crest Cell Contribution to the Developing Circulatory System

Cited by 281 publications

References 47 publications

Brg1 governs distinct pathways to direct multiple aspects of mammalian neural crest cell development

Brg1 governs distinct pathways to direct multiple aspects of mammalian neural crest cell development

Anomalous origin of the left pulmonary artery from the ascending aorta in two children with pulmonary atresia, subaortic ventricular septal defect and right-sided major aorto-pulmonary collateral arteries

Results of Screening for Intracranial Aneurysms in Patients with Coarctation of the Aorta

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