8 - 1 October 1, 2008 Lecture 8 Transmembrane and FACIT...

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Unformatted text preview: 1 October 1, 2008 Lecture 8 Transmembrane and FACIT collagens 2 (8) Oct 1 Transmembrane and FACIT collagens: skin blistering and cartilage defects Proteoglycans (9) Oct 6 Hyaruronan and chondrogenesis (10) Oct 8 Aggrecan and cartilage defects Perlecan: endochondral ossification and chondrodysplasis Oct 13 Thanksgiving University closed Oct 15 Term Test #1: collagens (11) Oct 20 Small leucine-rich proteoglycans (SLRPs): Decorin and biglycan and osteogenesis (12) Oct 22 Transmembrane proteoglycans: syndecan and growth factor signaling (13) Oct 27 Integrins and cell-matrix adhesion (14) Oct 29 Matrix remodeling metalloproteinases, ECM remodeling and cancer 3 Type Class alpha chain gene Length of collagenous domains (human) Main distribution Supramolecular assembly Genetic Disease XIII transmembrane 1(XIII) COL13A1 95+172+209 XXIII transmembrane 1(XXIII) COL23A1 186+75+111 XVII transmembrane 1(XVII) COL17A1 918 1 1 1 skin Hemi- desmosome Epidermolysis bullosa junctionalis mitis, generalized atrophic benign EB XXV transmembrane 1(XXV) COL25A1 44+238+189 1 1 1 brain XXVI 1(XXVI) COL26A1 69+33 testis, ovary JEB: Junctional epidermolysis bullosa Transmembrane Collagens: focus on collagen XVII 4 Powell etl al (2005) Exp Dermatology 30: 682-687 Electron microscopy of the cutaneous basement membrane zone emidesmosomes (HD) appear as rivet-like structures: multiprotein complexes that are critical for a stable connection between epithelial cells and the underlying basal lamina/ basement membrane 5 Anchoring fibrils Type VII collagen Simplex Hemi- desmosomal Junctional (JEB) Dystrophic EB (DEB) Epidermolysis Bullosa (EB) subclasses Basal keratinocytes Basement membrane Papillary dermis Schematic representation of anchoring fibrils as part of the anchoring complex at the DEJZ Dermal Epidermal Junction Zone Collagen XVII (also known as BP180/BPAG2) 6 Powell etl al (2005) Exp Dermatology 30: 682-687 Schematic representation of type XVII collagen (BP180/BPAG2) Hemidesmosomal plaque coiled-coil structures: ER helix assembly Type XVII collagen functions an an anchor protein Type IV collagen Laminin, Perlecan endodomain ectodomain 7 Anchoring Filaments Powell etl al (2005) Exp Dermatology 30: 682-687 Intracellular ligands Extracellular ligands Collagen XVII serves as an intracellular and extracellular anchor in the basement membrane zone schematic representation of the intra- and extracellular hemidesmosomal and basement membrane proteins that bind to type XVII collagen : www.oulu.fi/iho/project2.html 6 4 integrin Collagen XVII: binds to laminin-5 and 6 4 8...
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8 - 1 October 1, 2008 Lecture 8 Transmembrane and FACIT...

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