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Osteopetrosis due to lack of acid secretion by osteoclasts is characterized by abolished bone resorption, increased osteoclast numbers, but normal or even increased bone formation. In contrast, osteoclast-poor osteopetrosis appears to have less osteoblasts and reduced bone formation, indicating that...
May 18, 2014
Journal:
Calcif Tissue Int.
Author:
Thudium CS, Moscatelli I, Flores C, Thomsen JS, Brüel A, Gudmann Stæhr N, Hauge EM, Karsdal MA, Richter J, Henriksen K
Infantile malignant osteopetrosis (IMO) is a rare, lethal, autosomal recessive disorder characterized by non-functional osteoclasts. More than 50% of the patients have mutations in the TCIRG1 gene, encoding for a subunit of the osteoclast proton pump. The aim of this study was to restore the resorpt...
November 5, 2013
Journal:
Bone
Author:
Moscatelli I, Thudium CS, Flores C, Askmyr M, Gudmann NS, Andersen NM, Porras O, Karsdal MA, Villa A, Fasth A, Henriksen K, Richter J, Schulz AS
The osteoclast is vital for establishment of normal hematopoiesis in the developing animal. However, its role for maintenance of hematopoiesis in adulthood is more controversial. To shed more light on this process, we transplanted hematopoietic stem cells from two osteopetrotic mouse models, with la...
October 4, 2013
Journal:
Haematologica
Author:
Flores C, Moscatelli I, Thudium CS, Gudmann Stæhr N, Thomsen J, Brüel A, Karsdal MA, Henriksen K, Richter J
Infantile malignant osteopetrosis (IMO) is a rare, lethal, autosomal recessive disorder characterized by non-functional osteoclasts. More than 50% of the patients have mutations in the TCIRG1 gene, encoding for a subunit of the osteoclast proton pump. The aim of this study was to restore the resorpt...
July 29, 2013
Journal:
Bone
Author:
Moscatelli I, Thudium CS, Flores C, Askmyr M, Gudmann KN, Andersen NM, Porras O, Karsdal MA, Villa A, Fasth A, Henriksen K, Richter J, Schulz AS
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