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APPTT

PTAC/CFTR
Workshop

W32--Airway Differentiation, Pathogenesis, & Repair

Saturday, September 28, 2024
7:45 AM – 9:45 AM ET
Location:153 A-C

Recorded Session

The recent success of highly effective modulator therapies (HEMT) has transformed research in the CF field from focusing on rescuing mutant CFTR to identifying and addressing emergent challenges. A significant increase in life expectancy for individuals with CF is now accompanied by a higher predisposition to cancer (5to 7-fold vs non-CF population), as confirmed by recent large-scale studies. This presents a new challenge at the forefront of this disease.

To understand the mechanisms linking dysfunctional CFTR to carcinogenesis, we need to broaden our perspective beyond CF as merely a channelopathy and consider other cellular roles for CFTR. Besides transporting anions across epithelia, CFTR has been implicated in other functions. Indeed, it is emerging in the literature that CFTR is involved in essential cellular processes such as development, epithelial differentiation, maintenance of epithelial apical basolateral polarity, and tissue regeneration.

Another important question, relevant for gene therapy and gene editing, is identifying the "right" cells that normally express CFTR and determining whether the absence of functional CFTR disrupts the balance of epithelial cell types, particularly in the airways.

Learning Objectives:

  • Discuss the major non-ion channel defects occurring in CF airways.
  • Identify the airway submucosal glands progenitor source of ionocytes.
  • Comment on the novel role of ionocytes in the unpacking of mucins.
  • Understand how Notch signaling influences ionocyte precursor differentiation.
  • Cite a novel possible way of reducing goblet cell metaplasia.

Presentations:

  • 7:45 AM – 9:45 AM ET
    W32.1- Lef1 Induces Ionocyte Specification of Submucosal Gland Progenitors. Which One?

    Speaker: Weam S A Shahin, MD PhD – University of Iowa

  • 7:45 AM – 9:45 AM ET
    W32.2- Ionocytes localized in airway submucosal gland mucus tubule and ciliated ducts contribute to mucin unpacking

    Speaker: Amirala Ostad Mohammad Nazari, BSc (he/him/his) – Department of Anatomy, Physiology and Pharmacology, University of Saskatchewan, Saskatoon, SK, Canada

  • 7:45 AM – 9:45 AM ET
    W32.3- Temporal Dynamics in the Regulation of Pulmonary Ionocyte Development through NOTCH Signaling

    Speaker: Ruth-Love Damoah, n/a

  • 7:45 AM – 9:45 AM ET
    W32.4- ELD607, an Orai1 antagonist, reverses goblet cell metaplasia in βENaC mice.

    Speaker: Saira Ahmad, PhD – Eldec Pharmaceuticals