Comparison of the <i>ColIITg<sup>co</sup></i><sup><i>g</i></sup> phenotype with mouse models of MED, PSACH and MCDS.
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Phenotypic comparisons between the ColIITgcog, MED-causing Matn3 p.V194D (24, 26), PSACH-causing COMP p.T583M (25) and p.D469del (37), ColIITgrdw (40), MCDS-causing Col10a1 p.N617K and ColXTgcog mouse models (23, 33). Presence of ER stress was determined by evidence of increased levels of BiP (protein or mRNA). Mutant ECM protein secretion refers to any evidence showing secretion of mutant protein into the ECM contributing to an ECM dysfunction. Growth plate abnormalities include any dysplastic or disorganised growth plate architectures. All comparisons were made at 3 weeks of age unless stated otherwise. N/A—Not applied. VIF—Vascular invasion front; PZ—Proliferative zone; RZ—Resting zone. Comparison of the ColIITgcog phenotype with mouse models of MED, PSACH and MCDS.
