农学代谢性骨病分子细胞生物学.pptxVIP

代谢性骨病代谢性骨病是一类伴有钙、磷、维生素D代谢失调以及甲状旁腺功能、细胞因子与激素分泌异常的全身性骨骼病 [代谢性骨病的分类] 1.骨质疏松 2.佝偻-软骨病 3.原发性甲状旁腺机能亢进症 4.原发性甲状旁腺机能减退症 5.中毒性骨病(维生素D、氟、镉、铅、磷等中毒) 6.其他如粘多糖代谢异常，Marfan症，Paget症, SEDT-PA。osteoporosis：Represents a continuum, in which multiple pathogenetic mechanisms converge to cause loss of bone mass and microarchitectural deterioration of skeletal structure. These factors, coupled with an increased risk of falls, contribute to a high incidence of fragility fractures in osteoporotic patients.Skeletal fragility can result from: (a) failure to produce a skeleton of optimal mass and strength during growth; (b) excessive bone resorption resulting in decreased bone mass and microarchitectural deterioration of the skeleton; (c) an inadequate formation response to increased resorption during bone remodeling.The bone remodeling units or bone multicellular units (BMUs)Institute of Metabolism Endocrinology, Central South University骨组织的细胞成份成骨谱系：间充质干细胞，骨源性始组细胞（osteoprogenitor cells）、成骨细胞（osteoblast）、骨衬细胞（bone-lining cell）、骨细胞（osteocyte）破骨谱系：多潜能造血干细胞、破骨细胞（osteoclast）成软骨细胞、软骨细胞、破软骨细胞Institute of Metabolism Endocrinology, Central South University非细胞成分胶原非胶原蛋白骨矿物质血管神经Institute of Metabolism Endocrinology, Central South University组织细胞学基础Osteoblast development and differentiationTranscription factors Cbfa1/Runx2++++++?Surface markers STRO-1+++/SB-10/ALCAM00HOP-26/CD63++EnzymesAlkaline phosphatase++++++-Phex++++Collagen and non-collagenous proteinsCollagen type I?-+++++++-Osteocalcin +++-Bone sialoprotein--++- +++ +++ +++Osteopontin+?-/+-/+-/+- + +++ +++Effects of Wnt signaling on osseous cells. The canonical Wnt signaling pathway promotes the proliferation, expansion and survival of pre- and immature osteoblasts. Dkks, Sfrps, and Wif-1 antagonize Wnt signaling in osteoblasts to facilitate death of immature cells, but they may also downregulate the pathway in mature cells to induce terminal differentiation.Wnt signaling in osteoblasts and bone diseasesOsteoclast development and diffe