Expression of type VI collagen in the developing mouse heart

Klewer, Scott E.; Krob, Sonja L.; Kolker, Sandra J.; Kitten, Gregory T.

doi:10.1002/(sici)1097-0177(199803)211:3<248::aid-aja6>3.0.co;2-h

Cited by 59 publications

(31 citation statements)

References 26 publications

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“…In addition, there is abundant opportunity for remodeling of the ECM in the developing wall, because numerous matrix proteases are expressed at this stage of heart development. [62][63][64][65][66] Again, these spaces are continuous with the subepicardial space but do not connect with the lumen of the heart, and the endocardial lining is not compromised. In summary, remodeling of the myocardial ECM and production of signaling molecules are likely to play important roles in mesenchymal migration and vasculogenesis.…”

Section: Migration Of Vasculogenic Cells Within the Myocardiummentioning

confidence: 99%

Coronary Vessel Development

Wada

Willet

Bader

2003

ATVB

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Abstract-Development of the coronary vascular system is an interesting model in developmental biology with major implications for the clinical setting. Although coronary vessel development is a form of vasculogenesis followed by angiogenesis, this system uses several unique developmental processes not observed in the formation of other blood vessels. This review summarizes the literature that describes the development of the coronary system, highlighting the unique aspects of coronary vessel development. It should be noted that many of the basic mechanisms that govern vasculogenesis in other systems have not been analyzed in coronary vessel development. In addition, we present recent advances in the field that uncover the basic mechanisms regulating the generation of these blood vessels and identify areas in need of additional studies. A Brief Description of Coronary Vessel DevelopmentCoronary vessel development is an example of vasculogenesis followed by angiogenesis with unique variations specific to heart development. Vasculogenesis has been described as the de novo generation of blood vessels, whereas angiogenesis can be thought of as the generation of capillaries, veins, and arteries from preexisting vessels. The process begins with the delivery of vasculogenic cell types to the surface of the heart after beating has begun. These cells must then disperse throughout the heart; differentiate into endothelial cells, smooth muscle cells, pericytes, and fibroblasts; subsequently form arteries, veins, and capillaries; and finally connect to the aorta and coronary sinus. Delivery of a population of cells to an existing organ requires dynamic cellular events, and coordination of cell movement with the precise timing of delivery, commitment, and differentiation is critical for proper vessel formation and organ development. Originally, researchers thought that coronary vascular progenitors were derived from the cardiac mesoderm, like the other cell types in the myocardium and endocardium. 1,2 These data were first challenged by Manasek, 3 who demonstrated that the heart tube is initially composed of only endocardium and myocardium and that the epicardium arises from extracardiac regions. In more recent years, several studies have determined that progenitors of the epicardium and coronary vascular system are derived from extracardiac tissue, the proepicardial organ (PEO; Figure 1). Although the earliest location of these progenitors is still in debate, they appear to arise from splanchnic mesoderm. 4 -6 The PEO is associated with the septum transversum that grows from the dorsal body wall ventrally to divide the embryonic coelom into the pleuropericardial and peritoneal cavities and forms a portion of the diaphragm. 4,[7][8][9] However, the PEO is a transient structure that consists of a single epithelial layer folded into a shape resembling a grapelike cluster. In chicken (HH stage 17) and mouse (E9.5) embryos, the PEO migrates to and then over the surface of the heart to form the primitive epicardium (Figures 1 th...

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Section: Migration Of Vasculogenic Cells Within the Myocardiummentioning

confidence: 99%

Coronary Vessel Development

Wada

Willet

Bader

2003

ATVB

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“…This definition excludes genes located in the subtelomeric region such as S100␤, 27 as well as ADARB1, 28 collagen XVIII, 29 and collagen VI A1 and A2, which encode the alpha-1 and alpha-2 chains of collagen VI. 30 Collagen VI is a globular heterotrimer expressed in the basement membranes of endothelia and in the endocardial cushions during development, 31 which suggested it as a potential candidate for DS-CHD. 32 However, DUP21ZSC and two other cases in the literature 33 have complex CHD of the types associated with DS and have duplications which do not include collagen VI.…”

Section: Narrowing the Borders Of The Ds-chd Region The Telomeric Bormentioning

confidence: 99%

Down syndrome congenital heart disease: A narrowed region and a candidate gene

Barlow

Chen

Shi

et al. 2001

Genetics in Medicine

170

122

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Purpose: Down syndrome (DS) is a major cause of congenital heart disease (CHD) and the most frequent known cause of atrioventricular septal defects (AVSDs). Molecular studies of rare individuals with CHD and partial duplications of chromosome 21 established a candidate region that included D21S55 through the telomere. We now report human molecular and cardiac data that narrow the DS-CHD region, excluding two candidate regions, and propose DSCAM (Down syndrome cell adhesion molecule) as a candidate gene. Methods: A panel of 19 individuals with partial trisomy 21 was evaluated using quantitative Southern blot dosage analysis and fluorescence in situ hybridization (FISH) with subsets of 32 BACs spanning the region defined by D21S16 (21q11.2) through the telomere. These BACs span the molecular markers D21S55, ERG, ETS2, MX1/2, collagen XVIII and collagen VI A1/A2. Fourteen individuals are duplicated for the candidate region, of whom eight (57%) have the characteristic spectrum of DS-CHD. Results: Combining the results from these eight individuals suggests the candidate region for DS-CHD is demarcated by D21S3 (defined by ventricular septal defect), through PFKL (defined by tetralogy of Fallot). Conclusions: These data suggest that the presence of three copies of gene(s) from the region is sufficient for the production of subsets of DS-CHD. This region does not include genes located near D21S55, previously proposed as a "DS critical region," or the genes encoding collagens VI and XVIII. Of the potential gene candidates in the narrowed DS-CHD region, DSCAM is notable in that it encodes a cell adhesion molecule, spans more than 840 kb of the candidate region, and is expressed in the heart during cardiac development. Given these properties, we propose DSCAM as a candidate for DS-CHD. Genetics in Medicine, 2001:3(2):91-101.

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“…Molecular screening of families with trisomy 21 infants has demonstrated an association between genetic variations in the Col VI region and AV canal defects [Davies et al, 1995]. During heart development, Col VI is expressed within the developing AV canal in a pattern that parallels cell migration and valve remodeling [Kitten et al, 1996; Klewer et al, 1998]. Col VI is a component of the extracellular matrix that is speculated to link cells with the matrix through binding to cell surface integrins and structural matrix components [Aumailley et al, 1989; Aumailley et al, 1991].…”

Section: Introductionmentioning

confidence: 99%

Beta 1 integrin activation mediates adhesive differences between trisomy 21 and non‐trisomic fibroblasts on type VI collagen

Jongewaard¹,

Lauer²,

Behrendt³

et al. 2002

Am. J. Med. Genet.

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Trisomy 21 (Down syndrome) is a common genetic condition with a high incidence of congenital heart defects (CHD), particularly those involving abnormal development of the embryonic atrioventricular (AV) canal. Type VI collagen (Col VI) is expressed in the developing AV canal extracellular matrix, and has been associated with trisomy 21 AV canal defects in human genetic studies. Although the molecular mechanisms linking Col VI and trisomy 21 AV canal defects are not well understood, a computer model predicts increased cell adhesiveness is responsible for these CHD. We compared integrin-mediated cell adhesive properties for skin fibroblasts isolated from trisomy 21 and non-trisomic individuals on Col VI, fibronectin (FN) and type I collagen (Col I). Cell lines demonstrate similar adhesion profiles to FN and Col I, but all trisomy 21 cells display increased adhesive capacity for Col VI compared to non-trisomic fibroblasts. Cell adhesion to type VI collagen was shown to be GRGDS independent, but beta(1) integrin family dependent. Function-blocking antibodies identified alpha(3)beta(1) as the predominant integrin mediating trisomy 21 and non-trisomic skin fibroblast adhesion on Col VI. Trisomy 21 and non-trisomic fibroblasts display similar expression levels for each of the integrin receptors examined. A beta(1) integrin-activating antibody augments non-trisomic cell adhesion on Col VI, but has no effect upon trisomy 21 fibroblasts. These results demonstrate that beta(1) integrin family members mediate trisomy 21 and non-trisomic skin fibroblast adhesion for Col VI. Altered activation state of the beta(1) integrin is a mechanism responsible for increased trisomy 21 cell adhesion on Col VI.

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Expression of type VI collagen in the developing mouse heart

Cited by 59 publications

References 26 publications

Coronary Vessel Development

Coronary Vessel Development

Down syndrome congenital heart disease: A narrowed region and a candidate gene

Beta 1 integrin activation mediates adhesive differences between trisomy 21 and non‐trisomic fibroblasts on type VI collagen

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