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Preclinical rodent models for human bone disease, including a focus on cortical bone
Preclinical models (typically ovariectomized rats and genetically altered mice) have
underpinned much of what we know about skeletal biology. They have been pivotal for …
underpinned much of what we know about skeletal biology. They have been pivotal for …
Update on the genetics of osteogenesis imperfecta
Osteogenesis imperfecta (OI) is a heterogeneous heritable skeletal dysplasia characterized
by bone fragility and deformity, growth deficiency, and other secondary connective tissue …
by bone fragility and deformity, growth deficiency, and other secondary connective tissue …
Pathogenic mechanisms of osteogenesis imperfecta, evidence for classification
H Yu, C Li, H Wu, W **a, Y Wang, J Zhao… - Orphanet Journal of Rare …, 2023 - Springer
Osteogenesis imperfecta (OI) is a connective tissue disorder affecting the skeleton and other
organs, which has multiple genetic patterns, numerous causative genes, and complex …
organs, which has multiple genetic patterns, numerous causative genes, and complex …
Bone quality and mineralization and effects of treatment in osteogenesis imperfecta
BM Misof, N Fratzl-Zelman - Calcified tissue international, 2024 - Springer
Osteogenesis imperfecta (OI) is a rare congenital bone dysplasia characterized by high
fracture rates and broad variations in clinical manifestations ranging from mild to …
fracture rates and broad variations in clinical manifestations ranging from mild to …
Accelerated mineralization kinetics in children with osteogenesis imperfecta type 1
BM Misof, P Roschger, M Mähr, N Fratzl-Zelman… - Bone, 2023 - Elsevier
Not much is known about the time course of mineralization in newly formed bone from
healthy individuals nor in patients with bone disease. To investigate the early phase of …
healthy individuals nor in patients with bone disease. To investigate the early phase of …
Bone material properties in bone diseases affecting children
Abstract Purpose of Review Metabolic and genetic bone disorders affect not only bone mass
but often also the bone material, including degree of mineralization, matrix organization, and …
but often also the bone material, including degree of mineralization, matrix organization, and …
Evolution of primate interferon-induced transmembrane proteins (IFITMs): a story of gain and loss with a differentiation into a canonical cluster and IFITM retrogenes
Interferon-inducible transmembrane proteins (IFITMs) are a family of transmembrane
proteins. The subgroup of immunity-related (IR-) IFITMs is involved in adaptive and innate …
proteins. The subgroup of immunity-related (IR-) IFITMs is involved in adaptive and innate …
Mineralised bone properties in a child with recessive osteogenesis imperfecta type XIV and in a conditional Tmem38b knockout murine model (Runx2-Cre; …
Introduction OI type XIV is caused by variants in the TMEM38B gene, encoding for the
ubiquitously expressed endoplasmic reticulum trimeric intracellular cation channel type B …
ubiquitously expressed endoplasmic reticulum trimeric intracellular cation channel type B …
FiberO for an automated quantitative analysis of fibers orientation and organization in biological fibrous tissues
Many biological fibrous tissues exhibit distinctive mechanical properties arising from their
highly organized fibrous structure. In disease conditions, alterations in the primary …
highly organized fibrous structure. In disease conditions, alterations in the primary …
[HTML][HTML] 3D osteocyte lacunar morphometry of human bone biopsies with high resolution microCT: from monoclonal gammopathy to newly diagnosed multiple …
Osteocytes are mechanosensitive, bone-embedded cells which are connected via dendrites
in a lacuno-canalicular network and regulate bone resorption and formation balance …
in a lacuno-canalicular network and regulate bone resorption and formation balance …