Cell therapy of osteogenesis imperfecta



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Abstract

Genetically determinated type I collagen structure anomaly causes the group of innate diseases known as osteogenesis imperfecta. Type I collagen is the basic component of the bone tissue organic matrix and is produced by osteoblasts. The replacement cell therapy can be a radical treatment option for the osteogenesis imperfecta if the high stable osteoblast chimerism level is reached. Analogously with the classical hematopoietic stem cell transplantation the replacement of hypothetic osteogenic stem cells is necessary for the stable osteoblasts engraftment. Stem cells with the potency to skeletal tissues differentiation were first described by A.J. Friedenstein in the bone marrow stroma. The evolution of the stromal stem cells vision leaded to the “mesenchymal stem cells”, “stem cells of skeletal tissues” and even “medical signal cells” concepts appearance. The transplantation of all listed cell types can lead to the increase of the clinical status in patients with osteogenesis imperfecta which is temporary yet. There was no success in the achievement of the stable osteoblasts engraftment during experimental and clinical studies at the moment. Such fact could be associated with the deficiency of fundamental knowledge about the source osteoblasts origin in vivo. At the same time taking into account extremely low range and poor efficiency of current severe osteogenesis imperfecta forms therapy approaches the cell therapy with ex vivo cultivated bone marrow stromal cells application is quite promising.

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About the authors

V. S Sergeev

I.P. Pavlov First Saint Petersburg State Medical University.

Saint Petersburg, Russia

T. I Tichonenko

G.I. Turner Scientific Research Institute of Children's Orthopedics.

Saint Petersburg, Russia

D. S Buklaev

G.I. Turner Scientific Research Institute of Children's Orthopedics.

Saint Petersburg, Russia

A. G Baindurashvili

G.I. Turner Scientific Research Institute of Children's Orthopedics.

Saint Petersburg, Russia

B. V Afanasiev

I.P. Pavlov First Saint Petersburg State Medical University.

Saint Petersburg, Russia

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