A cartilage oligomeric matrix protein mutation associated with pseudoachondroplasia changes the structural and functional properties of the type 3 domain.

@article{Maddox2000ACO,
  title={A cartilage oligomeric matrix protein mutation associated with pseudoachondroplasia changes the structural and functional properties of the type 3 domain.},
  author={B. Kerry Maddox and Arati Mokashi and Doug R Keene and Hans Peter B{\"a}chinger},
  journal={The Journal of biological chemistry},
  year={2000},
  volume={275 15},
  pages={11412-7}
}
Cartilage oligomeric matrix protein (COMP) is a member of the thrombospondin family of extracellular matrix glycoproteins. All members of the family contain a highly conserved region of thrombospondin type 3 sequence repeats that bind calcium. A mutation in COMP previously identified in a patient with pseudoachondroplasia resulted in abnormal sequestration of COMP in distinctive rER vesicles. The mutation, Asp-446 --> Asn, is located in the type 3 repeats of the molecule. This region was… CONTINUE READING
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PSACH Mutation in COMP3 Peptides

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