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 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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