Lecture 6 Collagen IV and kidney filtration, anchoring fibrils and FACIT collagens Sept 28, 2016.pd

Lecture 6 Collagen IV and kidney filtration, anchoring fibrils and FACIT collagens Sept 28, 2016.pd

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1 Lecture Topics L1 Sept 12 Intracellular processing and secretion of ECM molecules L2 Sept 14 Molecular organization and classification of collagens: fibril-forming collagens. Type I collagen and connective tissue defects due to the improper assembly of type I collagen In the ER: scurvy and osteogenesis imperfecta. L3 Sept 19 Supramolecular assembly and cross-linking of type I collagen molecules. Dermatosparaxis disease L4 Sept 21 Connective tissue collagen defects: Ehlers-Danlos syndrome Basal laminae/basement membranes: network-forming type IV collagen L5 Sept 26 Role of basal laminae in epithelial morphogenesis: focus on collagen IV. L6 Sept 28 Role of collagen IV in kidney filtration Type VII collagens and anchoring fibrils in the dermal-epidermal junction zone and epidermolysis bullosa FACIT collagens and epiphyseal dysplasia Minicollagens and jelly fish nematocysts
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2 Kidney filtration: nephrons and the glomerular basement membrane (GMB)
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3 Figure 22-23 Molecular Biology of the Cell, 4 th Edition Electron micrograph of a cross section of a small capillary in a glomerulus of the kidney Glomerulus of kidney Sieve-like structure/fenestrae windows allows filtration of the blood
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4 Kidney filtration: nephrons and the glomerular basement membrane (GMB) Collagen IV and kidney filtration Defects in collagen IV and kidney diseases
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5 Schematic diagram representing the two distinct networks of type IV collagen found in GBM Embryonic GBM Adult GBM Interlinked by numerous disulfide bridges extensive that promote supercoiling of the network increased tensile strength protomer
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6 Alport Syndrome: a genetic defect in type IV collagen Alport syndrome is much more common in males because the gene that usually causes it (called COL4A5) is on the X chromosome. Women have two X chromosomes (XX), so they usually have a normal copy as well as an abnormal copy of the gene. Hence the disease is inherited from the mother. If the gene involved is COL4A3 and COL4A4, located on autosomes, then men and women are equally affected. Membranous glomerulonephritis
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7 renux.dmed.ed.ac.uk/.../AlportLong.html Normal GBM Alport Syndrome Thickening and 'falling apart' of Alport GBM. GBM This specialized basement membrane can be found in other parts of the body such as the inner ear and the part of the eye (lens) Alport Syndrome (Genetic Defect in Type IV Collagen/ hereditary nephritis) Kidney failure in early teens or twenties (End Stage Renal Disease-ESRD) Kidney deteriorates –blood in urine (hematuria) and high blood pressure develop Filtrate Filtrate
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8 The developmental switch from α 1(IV) 2 α 2(IV) to α 3(IV) α 4(IV) α 5(IV) is arrested in Alport syndrome by mutations in any of the genes encoding the a3(IV), a4(IV), or a5(IV) chains.
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