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Implementing Patch Clamp and Live Fluorescence Microscopy to Monitor Functional Properties of Freshly Isolated PKD Epithelium
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Calcium signaling in polycystic kidney disease- cell death and survival

Karla M Márquez-Nogueras1, Virdjinija Vuchkovska2, Ivana Y Kuo1

  • 1Department of Cell and Molecular Physiology, Stritch School of Medicine, Loyola University Chicago, 2160 S. First Ave, Maywood, IL, USA.

Cell Calcium
|April 6, 2023
PubMed

Insights

Polycystic kidney disease arises from mutations in polycystin proteins (PC-1 and PC-2), affecting calcium signaling. Recent research reveals how PC-1 and PC-2 structural insights clarify cell survival pathways in this condition.

Area of Science:

  • Nephrology
  • Molecular Biology
  • Genetics

Background:

  • Polycystic kidney disease (PKD) is characterized by kidney cysts and systemic complications like hypertension.
  • Loss-of-function mutations in polycystin 1 (PC-1) and polycystin 2 (PC-2) are the primary genetic cause of PKD.
  • Altered calcium signaling, partly due to PC-2's calcium permeability, is a key molecular feature, promoting cystic cell survival.

Approach:

  • This review synthesizes findings from the last five years.
  • It focuses on structural insights into PC-1 and PC-2.
  • The review examines how these insights illuminate calcium-dependent pathways regulating autophagy and the unfolded protein response.

Key Points:

  • Structural data on PC-1 and PC-2 provides a mechanistic understanding of PKD.
  • These proteins regulate crucial cell survival pathways, including autophagy and the unfolded protein response.
  • Calcium signaling is central to how polycystins influence cell fate in PKD.

Conclusions:

  • Understanding the structure-function relationship of polycystins is vital for deciphering PKD.
  • Targeting these calcium-dependent pathways may offer therapeutic strategies for PKD.
  • The interplay between polycystins, calcium, and cell survival pathways is critical in PKD pathogenesis.
Keywords:
ADPKDAutophagyCell survivalKidney cystsTRP channels

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