5 - Lecture 5 Extracellular Processing and Assembly of Type...

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1 Sept 22, 2008 Lecture 5 Extracellular Processing and Assembly of Type I Collagen Ehlers-Danlos Syndrome Basal Laminae/basement membranes
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2 Lecture Topics and Test Dates (1) Sept 8 Introduction (2) Sept 10 Intracellular processing and transport of ECM molecules Molecular organization of collagens, classification Fibrillar collagens: type I collagen - prototype (3) Sept 15 Connective tissue defects due to the improper assembly of type I collagen in the ER: scurvy and osteogenesis imperfecta (4) Sept 17 Extracellular processing and the supramolecular assembly of type I collagen Collagen defects: Ehlers-Danlos syndrome (EDS) (5) Sept 22 EDS continued Basal laminae/basement membranes: network-forming collagen IV {6) Sept 24 Role of basal laminae in epithelial morphogenesis and kidney filtration (7) Sept 29 Type VII collagens and anchoring fibrils in the dermal-epidermal junction zone
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3 Classical Type: highly elastic, velvety skin which may tear, bruise, or scar easily, joint hypermobility. Type I and V collagens Hypermobility Type: bruising, velvety-smooth skin, mildly hyperextensible skin,and loose, excessively loose joints. Type III collagen, tenascin X Vascular Type: fragile blood vessel walls and organ membranes, with a tendency to rupture or develop aneurysms. Type III collagen Kyphoscoliosis Type progressive deformity of spine-scoliosis, General joint looseness. Lysyl hydroxylase Arthrochalasia Type: very loose and unstable joints - recurrent partial or total dislocations Type 1 collagen Dematosparaxis Type: joints with extremely fragile skin which loses elasticity. ADAMTS2 EDS classifications http://en.wikipedia.org/wiki/Ehlers-Danlos_Syndrome
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4 The symptom cross-over for all types of EDS often leads to wrong or under diagnosis The focus of the lecture is on: 1) Classical Type: highly elastic, velvety skin which may tear, bruise, or scar easily. Joints are hyperextensible with a tendency to dislocate. 2)
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This note was uploaded on 09/29/2009 for the course CSB csb327 taught by Professor Ringuitte during the Fall '08 term at University of Toronto.

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5 - Lecture 5 Extracellular Processing and Assembly of Type...

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