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Updated: Aug 18, 2026

Visualization of Endoplasmic Reticulum Subdomains in Cultured Cells
Published on: February 18, 2014
Organelle acidification and disease
1Renal-Electrolyte Division, University of Pittsburgh School of Medicine, 3550 Terrace St., Pittsburgh, PA 15261, USA. weisz@pitt.edu
Abstract:
A subset of cellular compartments maintain acidic interior environments that are critical for the specific functions of each organelle and for cell growth and survival in general. The pH of each organelle reflects the balance between proton pumping, counterion conductance, and proton leak. Alterations in steady-state organelle pH due to defects in either proton pumping activity or counterion conductance have been suggested to contribute to the pathology of several diseases; however, definitive evidence remains elusive. This review describes recent evidence for the misregulation of organelle pH in the progression of cancer, Dent's disease, and cystic fibrosis.
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