Insight into the Formation and Regression of Brain AVMs from Notch4 Model of the Disease Relevant to HHT

Presenter

Wang, Rong PhDRong Wang, Ph.D. is a Professor and Mildred V. Strouss Endowed Chair in Vascular Surgery, in the Department of Surgery at the University of California, San Francisco. Dr. Wang had the distinction of working as a post-doctoral fellow in the laboratory of Michael Bishop, MD, who is a winner of the Nobel Prize in Medicine and Chancellor of UCSF.

Dr. Wang's team is engaged in state-of-the-art research involving key proteins necessary for blood vessel growth (angiogenesis) and arterial growth (arteriogenesis). They have found the Notch 4 protein can cause dramatic blood vessel enlargement in adult animals and the focal adhesion kinase protein is essential to maintain existing blood vessel structure. Her laboratory was the first to publish that Notch mutations can cause AVMs in mice, and correction of a causal molecular lesion can lead to reversal of the disease progression, opening a new line of inquiry linking this disease to genes crucial for arterial-venous differentiation.

Webinar

Join Dr. Rong Wang for a discussion of her recent discoveries presented at the 2015 International HHT Scientific Conference. These findings provide unprecedented insight uncovered from studying Brain AVM formation, and regression, in real time with high resolution microscopy in live animals with HHT. These studies may advance our understanding of how HHT disease is initiated and inspire new strategies in the development of treatment options.

Webinar Highlights

  • Explanation of notch signaling and its relevance to HHT
  • Research shows notch signaling is increased in human Brain AVMs
  • Notch and HHT2 (Alk1 or ACVRL1) share remarkable similarities
  • Discussion about AVM regression
  • Handout - What is Notch?   (an explanation provided by Dr. Christopher Hughes, Chairman, Cure HHT Global Research and Medical Advisory Board)

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Ask the Doctor: Dr. Felix Ratjen

Q: Can kids with HHT play sports? What are your recommendations?

Dr. Felix Ratjen, Toronto Pediatric HHT Center: Regular exercise is important for everybody, and this certainly includes children who have HHT. Most children with HHT can participate in sports at all levels including competitive sports, but there are a few exceptions. Contact sports are not recommended for children with HHT who have brain AVMs unless they are completely treated; you should talk to your doctor about that, if you are unsure what the status is. For children with untreated pulmonary AVMs, if their oxygen saturation is not normal, strenuous exercise may put them at extra risk and I usually recommend against it until treatment of the AVM has improved the situation. Even if the oxygen saturation is fine at rest, this may change during exercise. In cases where this is not clear, I perform a formal exercise test in the clinic. This also often provides comfort to parents as the test can determine the "safe" level of exercise that their child can participate in.