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Absence of FKBP10 in Recessive Type XI Osteogenesis Imperfecta Leads to Diminished Collagen Cross-Linking and Reduced Collagen Deposition in Extracellular Matrix

Recessive osteogenesis imperfecta (OI) is caused by defects in genes whose products interact with type I collagen for modification and/or folding. We identified a Palestinian pedigree with moderate and lethal forms of recessive OI caused by mutations in FKBP10 or PPIB, which encode endoplasmic retic...

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Bibliographische Detailangaben
Hauptverfasser: Barnes, Aileen M., Cabral, Wayne A., Weis, MaryAnn, Makareeva, Elena, Mertz, Edward L., Leikin, Sergey, Eyre, David, Trujillo, Carlos, Marini, Joan C.
Format: Artigo
Sprache:Inglês
Veröffentlicht: 2012
Schlagworte:
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC3470738/
https://ncbi.nlm.nih.gov/pubmed/22718341
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/humu.22139
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