Arteriovenous Differentiation Defects: AVM and HHT
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Questions and Answers

What is the role of Notch signaling in artery formation?

  • Essential for arteriovenous specification (correct)
  • Suppresses tip cell formation
  • Inhibits SMC recruitment
  • Promotes venous endothelial cell specification
  • What is the outcome of lineage tracing with venous endothelial cells in a mouse retina?

  • Specification of arterial endothelial cells
  • Formation of tip cells
  • Differentiation into smooth muscle cells
  • Contribution to artery formation (correct)
  • What is the consequence of defects in arteriovenous differentiation?

  • Hereditary Hemorrhagic Telangiectasia (correct)
  • Artery formation without SMC recruitment
  • Tip cell formation without Notch signaling
  • Venous endothelial cells specification
  • What is the role of flow-induced EC arterialization in artery formation?

    <p>Specifies arterial endothelial cells</p> Signup and view all the answers

    What is the function of tip cells in artery formation?

    <p>Contribute to artery formation</p> Signup and view all the answers

    What is the consequence of Notch inhibition in artery formation?

    <p>Inhibits arterial endothelial cell specification</p> Signup and view all the answers

    What is the characteristic of arterial endothelial cells?

    <p>They do not proliferate</p> Signup and view all the answers

    What is the primary function of arteries in the vascular system?

    <p>Transport oxygenated blood to the right heart</p> Signup and view all the answers

    Which structure is responsible for the transport of oxygenated blood to the left heart?

    <p>Vein</p> Signup and view all the answers

    In arteriovenous differentiation defects, what might be the consequence of abnormal vascular development?

    <p>Increased permeability of capillaries</p> Signup and view all the answers

    Which of the following cell types is not typically found in the venous endothelial cell lineage?

    <p>Arterial SMC</p> Signup and view all the answers

    What is the main characteristic feature of Hereditary Hemorrhagic Telangiectasia (HHT)?

    <p>Abnormal blood vessel formation</p> Signup and view all the answers

    In the context of arteriovenous malformations, which cell type plays a crucial role in the vascular integrity and function?

    <p>Endothelial cells</p> Signup and view all the answers

    What component of the vascular system is primarily responsible for brain neurovascular patterning?

    <p>Astrocyte</p> Signup and view all the answers

    What is the term for the process by which smooth muscle cells and pericytes cover the abluminal surface of the vessel?

    <p>Pericyte Coverage</p> Signup and view all the answers

    What is the name of the protein used as a marker for endothelial cells in the study?

    <p>CD31</p> Signup and view all the answers

    What is the name of the disease characterized by arteriovenous malformations and telangiectases?

    <p>Hereditary Hemorrhagic Telangiectasia</p> Signup and view all the answers

    In which study was the role of endothelial cell plasticity in flow-induced arterial-venous differentiation demonstrated?

    <p>Yuki Sato et al, PLOS ONE 2010</p> Signup and view all the answers

    What is the term for the space between the endothelium and the tunica media of an artery?

    <p>Perivascular space</p> Signup and view all the answers

    Which type of cell is responsible for the formation of the blood-brain barrier?

    <p>Astrocyte</p> Signup and view all the answers

    What is the name of the protein used as a marker for smooth muscle cells in the study?

    <p>αSMA</p> Signup and view all the answers

    What is the term for the process by which endothelial cells differentiate into arterial or venous endothelial cells?

    <p>Arteriovenous differentiation</p> Signup and view all the answers

    In which layer of the artery are smooth muscle cells and pericytes located?

    <p>Tunica media</p> Signup and view all the answers

    Which genetic disorder is associated with arteriovenous malformations in the lung, liver, and brain?

    <p>Hereditary Hemorrhagic Telangiectasia 2 (HHT2)</p> Signup and view all the answers

    What is a common symptom of Hereditary Hemorrhagic Telangiectasia (HHT) in individuals?

    <p>Nosebleeds</p> Signup and view all the answers

    Which marker is associated with the loss of capillary transition leading to arteriovenous differentiation defects?

    <p>Endothelial defects</p> Signup and view all the answers

    What is a consequence of late-stage brain arteriovenous malformations (bAVM)?

    <p>Neurological troubles</p> Signup and view all the answers

    What type of cell size change is observed in endothelial defects associated with arteriovenous differentiation defects?

    <p>Increase in cell size</p> Signup and view all the answers

    What is another name for Hereditary Hemorrhagic Telangiectasia (HHT) 5?

    <p>Rendu-Weber-Osler disease</p> Signup and view all the answers

    Which gene mutation is associated with the progression of brain arteriovenous malformations (bAVMs) during life?

    <p>2nd hit mutation</p> Signup and view all the answers

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