PUBLICATION

TMEM33 regulates intracellular calcium homeostasis in renal tubular epithelial cells

Authors
Arhatte, M., Gunaratne, G.S., El Boustany, C., Kuo, I.Y., Moro, C., Duprat, F., Plaisant, M., Duval, H., Li, D., Picard, N., Couvreux, A., Duranton, C., Rubera, I., Pagnotta, S., Lacas-Gervais, S., Ehrlich, B.E., Marchant, J.S., Savage, A.M., van Eeden, F.J.M., Wilkinson, R.N., Demolombe, S., Honoré, E., Patel, A.
ID
ZDB-PUB-190507-19
Date
2019
Source
Nature communications   10: 2024 (Journal)
Registered Authors
Wilkinson, Robert
Keywords
none
MeSH Terms
  • Acute Kidney Injury/genetics
  • Acute Kidney Injury/pathology*
  • Animals
  • Calcium/metabolism*
  • Cell Membrane/metabolism
  • Disease Models, Animal
  • Embryo, Nonmammalian
  • Endoplasmic Reticulum/metabolism
  • Endoplasmic Reticulum Stress
  • Epithelial Cells/cytology
  • Epithelial Cells/metabolism
  • Gene Knockdown Techniques
  • HeLa Cells
  • Humans
  • Kidney Tubules, Proximal/cytology
  • Kidney Tubules, Proximal/metabolism*
  • Lysosomes/metabolism
  • Membrane Proteins/genetics
  • Membrane Proteins/metabolism*
  • Membrane Proteins/physiology
  • Mice
  • Mice, Knockout
  • Mutation
  • Polycystic Kidney, Autosomal Dominant/genetics
  • Polycystic Kidney, Autosomal Dominant/pathology
  • RNA, Small Interfering/metabolism
  • TRPP Cation Channels/genetics
  • TRPP Cation Channels/metabolism*
  • TRPP Cation Channels/physiology
  • Zebrafish
  • Zebrafish Proteins/metabolism*
  • Zebrafish Proteins/physiology
PubMed
31048699 Full text @ Nat. Commun.
Abstract
Mutations in the polycystins cause autosomal dominant polycystic kidney disease (ADPKD). Here we show that transmembrane protein 33 (TMEM33) interacts with the ion channel polycystin-2 (PC2) at the endoplasmic reticulum (ER) membrane, enhancing its opening over the whole physiological calcium range in ER liposomes fused to planar bilayers. Consequently, TMEM33 reduces intracellular calcium content in a PC2-dependent manner, impairs lysosomal calcium refilling, causes cathepsins translocation, inhibition of autophagic flux upon ER stress, as well as sensitization to apoptosis. Invalidation of TMEM33 in the mouse exerts a potent protection against renal ER stress. By contrast, TMEM33 does not influence pkd2-dependent renal cystogenesis in the zebrafish. Together, our results identify a key role for TMEM33 in the regulation of intracellular calcium homeostasis of renal proximal convoluted tubule cells and establish a causal link between TMEM33 and acute kidney injury.
Genes / Markers
Figures
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Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Antibodies
Orthology
Engineered Foreign Genes
Mapping