BIOEN PRINC PHYS
BIOEN PRINC PHYS BIOEN 588
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This 18 page Class Notes was uploaded by Emily Braun II on Wednesday September 9, 2015. The Class Notes belongs to BIOEN 588 at University of Washington taught by Staff in Fall. Since its upload, it has received 40 views. For similar materials see /class/192000/bioen-588-university-of-washington in Bioengineering at University of Washington.
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Date Created: 09/09/15
BIOEN 588 Postmortem photo Postmortem xray image BIOEN 588 A decalcified bone Postmortem xray OI type II Human femur BIOEN 588 OSTEIOGENEIS IMPERFEOTA Likely mum m mmalnn Type I an quotmen BIOEN 588 Ammo acid sequence Tnp e hem Mmecul Mmecmar packmq Her rGwiPmrHyp G lyiproinyys4lyixivi Gem 10ka HER membrane C eavage of prepro sequences Structure Cmavaqe sue NHTtermmm domam CoHagan domam 300 mn Dlsmfide bands Pram Fruruz Proal CaHaqen he ix G obu ar regwon Conagen hkc scgmenl Nonhelmz segmem C eava 9 me COO H termmal domain Mamn G cNa DlsuHide bonds Lvsyl and proivl hydroxyrauon Hydraxvlvsvl glycosvlatian Heterosaccharide addu39mn KW N W Assembw of prucouagen lriple hel rx 7 C eavage 93 i W Pramlager amp 539 339 391 I I gt nRNAI4UkD v mRNA 5ka DAA Nuclear membrane W i 61751 mices Heterasacchande mvd ica on Cel msmbrane Sponlaneous self assemb y of cmlagen Biosynthesis BIOEN 588 Penttinen RP Lichtenstein JR Martin GR and McKusick VA 1975 Abnormal collagen metabolism in cultured cells in osteogenesis imperfecta Proc Natl Acad Sci U S A 72586 Barsh GS and Byers PH1981 Reduced secretion of structurally abnormal type procollagen in a form of osteogenesis imperfecta Proc Natl Acad Sci U S A 785142 Barsh GS David KE and Byers PH 1982 Type osteogenesis imperfecta a nonfunctional allele for pro alpha 1 l chains of type procollagen Proc Natl Acad Sci U S A 793838 Chu ML Williams CJ Pepe G Hirsch JL Prockop DJ Ramirez F 1983 Internal deletion in a collagen gene in a perinatal lethal form of osteogenesis imperfecta Nature 30478 BIOEN 588 Gly Gly Normal Abnormal G A T C G A T C BIOEN 588 O C New Accident BIOEN 588 OI IV C G Somatic Mosaicism BIOEN 588 OI II r OI II Gonadal Mosaicism O BIOEN 588 C NORMAL ALLELE OI A GENOMIC DNA BIOEN 588 Transmission electron micrograph BIOEN 588 0 COLLAGENS H Mum quotquot quotquot C L avaqe sue C eava 9 me NHTtermma domam Dlsmfide bands 0 Fr 7 CoHagan domam COOH termmal domain FY 11 Mamn G cNa Gal I m n 1 I y w sonawmsegnen mmnmm Nonhelmz segmem Procollagen Structure BIOEN 588 Frac on of prolem undigested 4 4 remuemnuve quotm Prowase Assay BIOEN 588 BIOEN 588 Hypothesis Cellular Pathology Based on Abnormal Procollagen StructureFunction BIOEN 588 proaiUL spL39 prooZUL C Oi OI C Medium Cells Abnormal procollagen is retained inside of 0 fibroblasts BIOEN 588 94gt 57gt on oooooo m J New Accident BIOEN 588 tax11am Transmission electron micrograph
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