Collagen and ER stress

Two cartilage collagen genes, the dysplasias their mutations cause, and why the short bones come from a stress response inside the cell rather than from a missing structural protein.

Collagen and ER stress: mutant chains cannot wind into threes and stay inside the endoplasmic reticulum, the cell answers with a stress response, and the cell is held flat instead of swelling and becoming bone. UPRno boneChains jamStress responseHeld flatnever pair upreprogrammes the cellno swelling, no bone

Collagen supplies the restraining network that aggrecan's swelling pressure pushes against (see Aggrecan and matrix sulfation). Both genes are SOX9 targets (see SOX9).

In this article

  • The mechanism is not a missing protein
  • The integrated stress response
  • ATF4 outside the stress response
  • Clearing the mutant protein
  • Compounds
  • Summary

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