3 - Sept 15, 2008 Lecture 3 Connective Tissue Defects Due...

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1 Sept 15, 2008 Lecture 3 Connective Tissue Defects Due To Improper Assembly of Type I Collagen in the ER and extracellular space: (1) Scurvy (nutritional deficiency) Continued & (2) Osteogenesis Imperfecta (OI) (brittle bone disease)
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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 α 2(I) α 1(I) α 1(I) Structural Organization of Type I Collagen N-propeptide C-propeptide α 1(I) 2 α 2(I) NC1 domain (Non-collagenous Domain 1) NC3 NC2 COL2 Extracellular space Procollagen COL1 Domain (Collagenous I domain) Comper (Ed) (1996) Extracellular Matrix, Vol2, Fig 2.4 Secretory pathway ER Procollagen
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4 Procollagen-I Comper (Ed) (1996) Extracellular Matrix, Vol2, 2-4 (modified) Extracellular processing of Type I collagen: cleavage of procollagen in the pericellular extracellular space Pro-collagen N-peptidase (ADAMT-2 and -3) C-terminal removal is critical for fibril assembly to begin tropocollagen Pro-collagen C-peptidase (BMP2 -an MMP) MMP: matrix metalloprotease ADAMT: a disintegrin and metalloprotease-transmembrane
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5 Proteolytically cleaved type I collagen that will assembly into fibrils ~ 15 diameter X 300 nm long – 1014 amino acids long collagenous domain Non-helical Telopeptide Telopeptides Tropocollagen Comper (Ed) (1996) Extracellular Matrix, Vol2, Fig 2.4 Flexible, rod-like molecule Surrounded by plasma membrane Extracellular compartment Diminished solubility Self-assembly
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6 Scurvy is a nutritional disease caused by deficiency of ascorbic acid (vitamin C). Scurvy
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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- Toronto.

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3 - Sept 15, 2008 Lecture 3 Connective Tissue Defects Due...

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