Ch26-Ortho

Ch26-Ortho - BONE JOINT SOFT TISSUE Modeling/RE-modeling...

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BONE JOINT SOFT TISSUE
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Modeling/RE-modeling
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CELLS of BONE OSTEOPROGENITOR (“STEM”) (TGFβ) OSTEOBLASTS (surface of spicule ), under control of calcitonin to take blood calcium and put it into bone. OSTEOCYTES (are osteoblasts which are now completely surrounded by bone) OSTEOCLASTS (macrophage lineage), under control of PTH to chew up the calcium of bone and put it into blood
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Proteins (organic) of BONE Type 1 collagen (90%) Cell adhesion proteins Calcium-binding proteins Proteins involved in mineralization Enzymes Growth factors GF-1, TGF-β, PDGF Cytokines Prostaglandins, IL-1, IL-6, RANKL Proteins Concentrated from Serum β2 –microglobulin Albumin IGF, insulin-like growth factor TGF, transforming growth factor PDGF, platelet-derived growth factor IL, interleukin RANKL, RANK ligand
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Minerals (IN-organic) of BONE HYDROXY-APATITE Ca5(PO4)3(OH) Ca10(PO4)6(OH)2
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ADJECTIVES of BONE Compact Dense Cortical Spongy Cancellous Membranous Endosteal Woven Lamellar Spicular
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Woven vs. “Lamellar”
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-BLASTS/-CLASTS Ca++ PTH Ca++ Calcitonin
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BONE DISEASES 1) MALFORMATIONS AND DISEASES CAUSED BY DEFECTS IN NUCLEAR PROTEINS AND TRANSCRIPTION FACTORS, polydactyly, syndactyly, absence of a bone 2) DISEASES CAUSED BY DEFECTS IN HORMONES AND SIGNAL TRANSDUCTION MECHANISMS, achondroplasia, thanatophoria 3) DISEASES ASSOCIATED WITH DEFECTS IN EXTRACELLULAR STRUCTURAL PROTEINS Type 1 Collagen Diseases (Osteogenesis Imperfecta) Types 2, 10, and 11 Collagen Diseases 4) DISEASES ASSOCIATED WITH DEFECTS IN FOLDING AND DEGRADATION OF MACROMOLECULES Mucopolysaccharidoses 5) DISEASES ASSOCIATED WITH DEFECTS IN METABOLIC PATHWAYS (ENZYMES, ION CHANNELS, AND TRANSPORTERS) Osteopetrosis 6) DISEASES ASSOCIATED WITH DECREASED BONE MASS Osteoporosis 7) DISEASES CAUSED BY OSTEOCLAST DYSFUNCTION Paget Disease (Osteitis Deformans) 8) DISEASES ASSOCIATED WITH ABNORMAL MINERAL HOMEOSTASIS Ricketts and Osteomalacia Hyperparathyroidism Renal Osteodystrophy
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1) MALFORMATIONS AND DISEASES CAUSED BY DEFECTS IN NUCLEAR PROTEINS AND TRANSCRIPTION FACTORS Congenital absence of a, usually single, bone: phalanx, rib, clavicle Supernumerary digit (polydactyly) Syndactyly CRANIORACHISCHISIS
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2) DISEASES CAUSED BY DEFECTS IN HORMONES AND SIGNAL TRANSDUCTION MECHANISMS Achondroplasia, dwarf (non-lethal) Thanatophoria, dwarf (lethal, FGF-3 mutations) a point mutation (usually Arg for Gly375) in the gene that codes for FGF receptor 3 (FGFR3), which is located on the short arm of chromosome 4. In the normal growth plate, activation of FGFR3 inhibits cartilage proliferation; A MUTATION causes FGFR3 to be constantly activated.
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Short arms and extra folds of skin Achondroplastic “dwarf” Thanatophoric “dwarf”, often lethal
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3) DISEASES ASSOCIATED WITH DEFECTS IN EXTRACELLULAR STRUCTURAL PROTEINS
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This note was uploaded on 01/25/2012 for the course PDBIO 305 taught by Professor Woods,a during the Fall '08 term at BYU.

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Ch26-Ortho - BONE JOINT SOFT TISSUE Modeling/RE-modeling...

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