title: Stem cells and bone diseases: new tools, new perspective.
abstract: Postnatal skeletal stem cells are a unique class of progenitors with biological properties that extend well beyond the limits of stemness as commonly defined. Skeletal stem cells sustain skeletal tissue homeostasis, organize and maintain the complex architectural structure of the bone marrow microenvironment and provide a niche for hematopoietic progenitor cells. The identification of stem cells in the human post-natal skeleton has profoundly changed our approach to the physiology and pathology of this system. Skeletal diseases have been long interpreted essentially in terms of defective function of differentiated cells and/or abnormal turnover of the matrix that they produce. The notion of a skeletal stem cell has brought forth multiple, novel concepts in skeletal biology that provide potential alternative concepts. At the same time, the recognition of the complex functions played by skeletal progenitors, such as the structural and functional organization of the bone marrow, has provided an innovative, unifying perspective for understanding bone and bone marrow changes simultaneously occurring in many disorders. Finally, the possibility to isolate and highly enrich for skeletal progenitors, enables us to reproduce perfectly normal or pathological organ miniatures. These, in turn, provide suitable models to investigate and manipulate the pathogenetic mechanisms of many genetic and non-genetic skeletal diseases. This article is part of a Special Issue entitled Stem cells and Bone.
authors.affiliation: Department of Molecular Medicine, Sapienza University of Rome, Italy. Electronic address: mara.riminucci@uniroma1.it.
authors.forename: Mara
authors.initials: M
authors.lastname: Riminucci
authors.affiliation: Department of Molecular Medicine, Sapienza University of Rome, Italy.
authors.forename: Cristina
authors.initials: C
authors.lastname: Remoli
authors.affiliation: Craniofacial and Skeletal Diseases Branch, National Institute of Craniofacial and Dental Research, National Institutes of Health, Department of Health and Human Services, Bethesda, MD, USA.
authors.forename: Pamela G
authors.initials: PG
authors.lastname: Robey
authors.affiliation: Department of Molecular Medicine, Sapienza University of Rome, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Bianco
mesh_terms: D001847:Bone Diseases / Q000235:genetics / Q000473:pathology*; D001859:Bone Neoplasms / Q000473:pathology / Q000556:secondary; D019337:Hematologic Neoplasms / Q000473:pathology; D006801:Humans; D013234:Stem Cells / Q000166:cytology*; D059016:Tumor Microenvironment
keywords: Bone diseases; Bone metastasis; Fibrous dysplasia; Hematopoietic cancer; Hematopoietic niche; Skeletal stem cells