Col1A-2 Mutation in Osteogenesis Imperfecta Mice Contributes to Long Bone Fragility by Modifying Cell-Matrix Organization

Abstract

Osteogenesis imperfecta (OI) is a rare congenital bone dysplasia generally caused by a mutation of one of the type I collagen genes and characterized by low bone mass, numerous fractures, and bone deformities. The collagen organization and osteocyte lacuna arrangement were investigated in the long bones of 17-week-old wildtype (WT, n = 17) and osteogenesis imperfecta mice (OIM, n = 16) that is a validated model of severe human OI in order to assess their possible role in bone fragility. Fractures were counted after in vivo scanning at weeks 5, 11, and 17. Humerus, femur, and tibia diaphyses from both groups were analyzed ex vivo with pQCT, polarized and ordinary light histology, and Nano-CT. The fractures observed in the OIM were more numerous in the humerus and femur than in the tibia, whereas the quantitative bone parameters were altered in different ways among these bones. Collagen fiber organization appeared disrupted, with a lower birefringence in OIM than WT bones, whereas the osteocyte lacunae were more numerous, more spherical, and not aligned in a lamellar pattern. These modifications, which are typical of immature and less mechanically competent bone, attest to the reciprocal alteration of collagen matrix and osteocyte lacuna organization in the OIM, thereby contributing to bone fragility.

Growth and mortality model of Chacunda gizzard shad Anodontostoma chacunda (Bleeker, 1852) in Tarakan waters, North Kalimantan, Indonesia

“…According to Agustiana et al (2023), the protein concentration from A. chacunda has the best base material of ash and carbohydrate content. A drawback of this fish is its numerous bones (André et al 2023), which are similar to those found in the fish Puput (Ilisha elongata) (Liu et al 2022), where I. elongata and A. chacunda have something in common, namely numerous bones and a flat shape (Firdaus and Salim 2011). The ecological habitat of A. chacunda is located in coastal waters (Amran and Daming 2023) and estuaries (Salim et al 2022), with a substantial population feeding primarily on benthic organisms (Barrera-Oro et al 2019) and Bacillariophyceae (Wang et al 2022) detritus (Liu and Yao 2023), thus this species categorized as omnivores and pelagic feeders (Hein et al 2018).…”