Unique Matrix Structure in the Rough Endoplasmic Reticulum Cisternae of Pseudoachondroplasia Chondrocytes

Mutations in cartilage oligomeric matrix protein (COMP) cause two skeletal dysplasias, pseudoachondroplasia (PSACH) and multiple epiphyseal dysplasia (MED/EDM1). Because COMP exists as a homopentamer, only one mutant COMP subunit may result in an abnormal complex that is accumulated in expanded roug...

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Bibliographische Detailangaben
Hauptverfasser: Merritt, Thomas M., Bick, Roger, Poindexter, Brian J., Alcorn, Joseph L., Hecht, Jacqueline T.
Format: Artigo
Sprache:Inglês
Veröffentlicht: American Society for Investigative Pathology 2007
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Regular Articles
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC1762700/
https://ncbi.nlm.nih.gov/pubmed/17200202
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.2353/ajpath.2007.060530
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