Collagen XVIII in tissue homeostasis and dysregulation — Lessons learned from model organisms and human patients

• Published in: Matrix biology Vol. 57-58; no. 1; pp. 55 - 75
• Main Authors: Heljasvaara, Ritva, Aikio, Mari, Ruotsalainen, Heli, Pihlajaniemi, Taina
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 01.01.2017; Elsevier Science Ltd
• Subjects: Angiogenesis; Animal models; Animals; Basement membrane; Basement Membrane - drug effects; Basement Membrane - metabolism; Basement Membrane - pathology; C-Terminus; Cell culture; Cell migration; Cell Movement - drug effects; Cell Proliferation - drug effects; Cell survival; Collagen; Collagen (type XVIII); Collagen Type VIII - genetics; Collagen Type VIII - metabolism; Encephalocele; Encephalocele - genetics; Encephalocele - metabolism; Encephalocele - pathology; Endostatin; Endostatins - pharmacology; Endothelial cells; Endothelial Cells - drug effects; Frizzled protein; Gene Expression Regulation; Genetic disorders; Heparan sulphate proteoglycan; Homeostasis; Homeostasis - genetics; Humans; Isoforms; Knobloch syndrome; Laminin; Membranes; Multiplexin; Mutation; Neoplasms - genetics; Neoplasms - metabolism; Neoplasms - pathology; Neovascularization, Pathologic - genetics; Neovascularization, Pathologic - metabolism; Neovascularization, Pathologic - pathology; Neovascularization, Pathologic - prevention & control; Progenitor cells; Protein Domains; Protein Isoforms - genetics; Protein Isoforms - metabolism; Proteolysis; Rare diseases; Recombinant Proteins - pharmacology; Retinal Detachment - congenital; Retinal Detachment - genetics; Retinal Detachment - metabolism; Retinal Detachment - pathology; Thrombospondin; Tumors; Wnt protein; Tsp-1; LM; RPE; CNS; BM; HS; ILM; Aβ; Knobloch syndrome; GBM; DUF; SNP; SPR; CSF; ApoE; Multiplexin; AD; HSPG; HCC; MW; NMJ; GAG; ECM; Basement membrane; CS; MMP; CLE-1; Fz; NC; Heparan sulphate proteoglycan; LH

Collagen XVIII is a ubiquitous basement membrane (BM) proteoglycan produced in three tissue-specific isoforms that differ in their N-terminal non-collagenous sequences, but share collagenous and C-terminal non-collagenous domains. The collagenous domain provides flexibility to the large collagen XVI...