• Seminar
  • BCRT

Seminar @Cranach House | Franklin Lo: "Aortic 'Disease-In-A-Dish': Lineage-Specific Phenotypic Abnormalities in iPSC-Derived Smooth Muscle Cells From Loeys-Dietz Syndrome"

Lecture: "Aortic 'Disease-In-A-Dish': Lineage-Specific Phenotypic Abnormalities in iPSC-Derived Smooth Muscle Cells From Loeys-Dietz Syndrome"

Aortic Aneurysms are characterised by a dilatation of the aorta due to the weakening of the blood vessel wall. They can lead to life-threatening aortic dissection or rupture if the aneurysm reaches a critical size.  Patients with Loeys-Dietz syndrome (LDS), a genetic connective tissue disorder caused by mutations in the components of TGF-β signalling pathway, have a high risk for thoracic aortic aneurysms (TAAs). With a life expectancy of just 37 years and no available therapies to prevent or cure TAAs, there is a huge unmet clinical need for patients with LDS.

In his PhD research, he aimed to understand the underlying mechanisms driving TAA formation in LDS patients.  Smooth muscle cells (SMCs) are hypothesised to play a key role in this process. Importantly, the aorta consists of SMCs derived from different embryonic origins, and these lineage-specific cells show varying susceptibility to vascular diseases. Therefore, his research focused on using patient-derived induced pluripotent stem cells (iPSCs) to generate lineage-specific SMCs to investigate their roles in the development of TAAs in LDS. The aim is to identify therapeutic targets that can lead to the development of new therapeutics for this devastating condition. 

Overview of the Talk

  • Overview of the Talk

    The presentation will focus on the current research in disease modeling of thoracic aortic aneurysms (TAAs) associated with Loeys-Dietz syndrome (LDS). Using induced pluripotent stem cells derived from LDS patients, the talk will highlight the role of lineage-specific smooth muscle cells in the pathogenesis of TAAs and explore potential therapeutic targets to address this fatal condition.

    Speech Plan:

    1. Introduction 

    • Overview of Loeys-Dietz Syndrome (LDS)
    • Clinical challenges associated with TAAs in LDS patients

    2. Research Overview 

    • Use of iPSC technology to study LDS
    • Importance of lineage-specific differences in SMCs and susceptibility to aortic diseases

    3. Mechanisms Underlying TAA Development

    • Findings from iPSC-derived SMCs in LDS models
    • How lineage-specific SMCs contribute to the formation of TAAs

    4. Therapeutic Implications and Future Directions 

    • Identification of potential therapeutic targets
    • Strategies for the development of drugs to slow or prevent TAA formation
    • Future steps in translating iPSC models into clinical applications

    5. Conclusion

About the Speaker

Franklin Lo obtained a BSc in Regenerative Medicine from the University of Edinburgh (2020). He then secured a British Heart Foundation-funded MRes + PhD Studentship in Cardiovascular Research at the University of Cambridge. During his MRes (2021), Lo worked on aortic aneurysms, atherosclerosis, and cardiac ischaemic reperfusion injury. His current PhD research focuses on disease modelling of aortic aneurysms using iPSCs in Prof Sanjay Sinha’s lab at the Cambridge Centre of Stem Cell Institute. 

Info

When
November 6, 2024
01:00 - 02:00 pm

Where
BIH Center for Regenerative Therapies (BCRT)
Cranach Haus | Auditorium 0.0044/45
Föhrer Str. 15
13353 Berlin