To identify mutations that cause hereditary hemorrhagic telangiectasia (HHT, or Rendu-Osler-Weber syndrome), clinical evaluations and genetic studies were performed on 32 families. Linkage studies in four of eight families indicated an endoglin (ENG) gene mutation. ENG sequences of affected members of the four linked families and probands from the 24 small families were screened for mutations, ...

The solution to the canonical problem of a radiating infinitesimal electric dipole antenna that is centred in a multilayered, concentric metamaterial-based spherical shell system is presented. It is demonstrated that when this system is electrically small, a specifically designed homogenous and isotropic epsilon-negative (ENG) layer can function as a distributed matching element to the antenna ...

OBJECTIVE To determine if angiogenesis is altered in adult Endoglin heterozygous (Eng(+/-)) mice, the animal model for the vascular disorder hereditary hemorrhagic telangiectasia type 1 (HHT1). METHODS Primary cultures of endothelial cells were generated from Eng(+/-) and Eng(+/+) mice and analyzed for proliferation, migration, and ability to form capillary-like tubes. Endothelial cells deriv...

Endoglin (ENG), an ancillary receptor for several members of the transforming growth factor (TGF)-beta superfamily, has a well-studied role in endothelial function. Here, we report that endoglin also plays an important role early in development at the level of the hemangioblast, an embryonic progenitor of the hematopoietic and endothelial lineages. Eng(-/-), Eng(+/-) and Eng(+/+) mouse embryoni...

BACKGROUND Renal ischaemia-reperfusion (I-R) can cause acute tubular necrosis and chronic renal deterioration. Endoglin, an accessory receptor for Transforming Growth Factor-beta1 (TGF-beta1), is expressed on activated endothelium during macrophage maturation and implicated in the control of fibrosis, angiogenesis and inflammation. METHODS Endoglin expression was monitored over 14 days after ...

BACKGROUND AND PURPOSE Endoglin deficiency causes hereditary hemorrhagic telangiectasia-1 and impairs myocardial repair. Pulmonary arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia-1 are associated with a high incidence of paradoxical embolism in the cerebral circulation and ischemic brain injury. We hypothesized that endoglin deficiency impairs stroke recovery....

BACKGROUND AND PURPOSE A 6-base insertion (6bINS) polymorphism in intron 7 of the endoglin gene (ENG), which codes for a component of the transforming growth factor-beta receptor complex, was reported to be associated with intracranial aneurysm (IA) in a Japanese population. A recent report using a white population could not replicate the association. We tested for this association with high st...

Endoglin is a TGF-beta superfamily receptor critical for endothelial cell function. Mutations in this gene are associated with hereditary hemorrhagic telangiectasia type I (HHT1), and clinical signs of disease are generally more evident later in life. We previously showed that systemic vessels of adult Eng heterozygous (Eng(+/-)) mice exhibit increased vasorelaxation due to uncoupling of endoth...

1Associate Member of ASCE, Ph.D., Assoc. Professor, Dept. of Civil and Env. Eng., Stanford Univ., and Director, Center for Integrated Facility Engineering (Terman Eng. Ctr., 380 Panama Mall, Stanford, CA 94305-4020, USA) E-mail:[email protected] 2Ph.D., Sr. Research Scientist, Dept. of Civil and Env. Eng., Stanford Univ. and Executive Director, Center for Integrated Facility Engineering (CIF...

Electrical Substrate Elimination in 135 Consecutive Patients With Brugada Syndrome Carlo Pappone, MD, PhD; Josep Brugada, MD, PhD; Gabriele Vicedomini, MD; Giuseppe Ciconte, MD; Francesco Manguso, MD, PhD; Massimo Saviano, MD; Raffaele Vitale, MD; Amarild Cuko, MD; Luigi Giannelli, MD; Zarko Calovic, MD; Manuel Conti, MD; Paolo Pozzi, Eng; Andrea Natalizia, PhD, Eng; Simonetta Crisà, Eng; Valer...