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Journal article

Unique matrix structure in the rough endoplasmic reticulum cisternae of pseudoachondroplasia chondrocytes.

Abstract:

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 rough endoplasmic reticulum (rER) cisternae, a hallmark of PSACH. Type IX collagen and matrilin-3 (MATN3), also accumulate in the rER cisternae of PSACH chondrocytes, but it is unknown how mutant COMP ...

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Publication status:
Published

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Publisher copy:
10.2353/ajpath.2007.060530

Authors


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Institution:
University of Oxford
Department:
Oxford, MSD, Physiology Anatomy and Genetics
Role:
Author
Journal:
The American journal of pathology
Volume:
170
Issue:
1
Pages:
293-300
Publication date:
2007-01-05
DOI:
EISSN:
1525-2191
ISSN:
0002-9440
URN:
uuid:e8fe6342-fdcb-42b7-916d-67306a07f9e6
Source identifiers:
485153
Local pid:
pubs:485153

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