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Osteopetrosis Mutation R444L Causes Endoplasmic Reticulum Retention and Misprocessing of Vacuolar H(+)-ATPase a3 Subunit

Osteopetrosis is a genetic bone disease characterized by increased bone density and fragility. The R444L missense mutation in the human V-ATPase a3 subunit (TCIRG1) is one of several known mutations in a3 and other proteins that can cause this disease. The autosomal recessive R444L mutation results...

Ausführliche Beschreibung

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Bibliographische Detailangaben
Hauptverfasser: Bhargava, Ajay, Voronov, Irina, Wang, Yongqiang, Glogauer, Michael, Kartner, Norbert, Manolson, Morris F.
Format: Artigo
Sprache:Inglês
Veröffentlicht: American Society for Biochemistry and Molecular Biology 2012
Schlagworte:
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC3411020/
https://ncbi.nlm.nih.gov/pubmed/22685294
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M112.345702
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