2005
DOI: 10.2741/1750
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Human osteopetroses and the osteoclast V-H+-ATPase acidification system

Abstract: Osteopetroses are a heterogeneous group of human genetic diseases characterized by generalized increase in bone density due either to a decreased osteoclast population, defect in osteoclast function, or both. Current knowledge of the pathogenesis suggests defects that may be either intrinsic to osteoclast-monocyte lineage or extrinsic to the mesenchymal cells that support osteoblast ontogeny and activation. Four clinically distinct forms of human osteopetroses currently recognized are the infantile malignant a… Show more

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Cited by 12 publications
(9 citation statements)
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“…The a3 subunit is a core protein in the multimeric structure of the osteoclastic V-ATPase (Ogbureke et al 2005;Xu et al 2007).…”
Section: The A3 Subunit Of the V-atpasementioning
confidence: 99%
See 1 more Smart Citation
“…The a3 subunit is a core protein in the multimeric structure of the osteoclastic V-ATPase (Ogbureke et al 2005;Xu et al 2007).…”
Section: The A3 Subunit Of the V-atpasementioning
confidence: 99%
“…There are numerous animal models of osteopetrosis and we refer to previous reviews for a detailed description (Ogbureke et al 2005;Van Wesenbeeck and Van Hul 2005). These models can be broadly separated into models with osteoclasts and models without osteoclasts.…”
Section: Animal Models Of Osteopetrosismentioning
confidence: 99%
“…The protein responsible for chloride transport is the product of the ClC7 gene [40]. Key members of the multimolecular ATPase complex include the a3 and d2 subunits [41,42] and Ac45, with the latter facilitating movement of vesicles to the cell surface [43]. The same role has been ascribed to osteoclast specific transmembrane protein 1 (OSTM1) [44], the product of the gl gene, mutation of which results in severe infantile osteopetrosis [45].…”
Section: Overview Of Osteoclast Functionmentioning
confidence: 95%
“…Subunit a4 is found in the kidney (65). Mutations in subunit a3 cause autosomal malignant osteopetrosis (67–69).…”
Section: V‐atpasesmentioning
confidence: 99%