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Med Micro ECM

Extracellular Matrix Components/Funtion

What are the four major components of the extracellular matrix? collagens, elastin, proteoglycans, and hyaluronans
What is the structure of collagen? primary subunits are left handed helices; these associate into right handed triple helices known as tropocollagens, which are bundled together to form collagen fibers
What is the structure of elastin? Tropoelastin polypeptides are synthesized in the rough ER and secreted to the extracellular space. Once there, they congretate to a scaffold of fibrillin
What is the structure of proteoglycan? glycosaminoglycans (GAGs) bound to a core protein; made of modified disaccharides
What is the function of collagen? provides tensile strength by resisting stretching
What is the function of elastin? provides tissues with elasticity, the ability to return to their original size and shape
What is the function of proteoglycan? help anchor cells to the basal lamina through crosslinking with other components of the ECM (sometimes collagen); sometimes function as regulators (such as heparin) as well
Why are prolines important in collagen? They contort the polypeptide backbone and forces it into a left handed helix
Why are glycines important in collagen? Their amino acid side chain is simply a hydrogen atom. This allows the helix to form. Larger side groups would cause steric hindrance.
What is fibrosis? excess collagen (build up of scar tissue)
What causes scurvy? lack of vitamin C which causes a decreased hydroxylation of proline residues
Ehler's-Danlos Syndrome is a result of what? underproduction or incomplete processing of different collagens
Osteogenesis imperfecta (brittle-bone syndrome) is cause by... a mutation of type I collagen; this interferes with triple helix assembly
What amino acid crosslinking is seen in elastin? How many are bound together? lysine; four
Is elastin a bundle or a mesh network? mesh network
What gives elastin the ability to "snap back"? interspersed hydrophobic and hydrophilic domains
What condition are homozygous alpha1-AT mutants predisposed to? Why? emphysema; because the elastin in the alveolar walls is degraded due to inhibition of elastase
Fibrillin mutations result in... Marfan's syndrome
Where are proteoglycans often found? plasma membrane
What do proteoglycans sequester? growth factors
What are hyaluronans? glycosaminoglycans (GAGs that are not bound to the core protein as is seen in proteoglycans) that interact with water molecules to form hydration spheres
What is the function of hyaluronans? resist compression and serve as lubricants for joints
What is the function of aggrecans? resistance of compression
Where are aggrecans most prevalent? cartilage
What are the ECM crosslinking proteins? lamin and fibernectin
Created by: smuncy



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