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Extracellular matrix

WVSOM Class of 2012 Extracellular Matrix

what is the function of the extracellular matrix? tissue resiliency and cellular interactions
where is the extracellular matrix concentrated? connective tissues
how many different kinds of collagen are in the body? 14
what is the most abundant protein of the body, making up half our protein by weight? collagen
which direction is the helix of collagen? left-handed (opposite of the norm)
what elements provide tensile strength by resisting stretching forces? collagen
what is every 3rd residue of an α-chain? glycines
About 10% of the total weight of collagen represents sugar side chains, mostly glucose-galactose disaccharides. What process do they contribute to? glycosylation
Hydroxyl (OH) groups are bound to numerous proline and lysine residues of collagen. What process do they contribute to? hydroxylation
what molecule crosslinks the lysyl residues at the end of the collagen fibers? lysyl oxidase
why do muscles look striated? from the pattern of overlap between tropocollagens
why does the collagen process move outside of the cell? it is too large to remain inside the cell
Excessive collagen production (e.g. pulmonary fibrosis, atherosclerosis, scar tissue in liver due to over consumption of alcohol) are what collagen defect? fibrosis
Lack of hydroxyproline residues due to dietary deficiency of vitamin C leads to what collagen defect? scurvy
what are the symptoms of scurvy? Symptoms include weak and malformed bones, teeth, skin, blood vessel walls, and dermal hemorrhaging
Mutations resulting in underproduction or incomplete processing of different collagens leads to what collagen defect? Ehlers-Danlos Syndrome (rubber-man syndrome
what are the symptoms of Ehlers-Danlos Syndrome (rubber-man syndrome)? loose skin and joints or neonatal death.
Mutations of type I collagen, which interfere with triple helix assembly (e.g. substitution of glycines residues with more bulky amino acids). The most severe mutations result in lethality in utero or soon after birth is what collagen deficiency? Osteogenesis imperfecta (brittle-bone syndrome)
what component provides tissues with elasticity, the ability to return to their original size and shape? elastin
elastin is abundant in what structures? ligaments, arteries, aortic arch, some in tendons, skin, and loose connective tissue
what are the major components of the extracellular matrix? collagen, elastin, hyaluronan and proteoglycans
polypeptides that are produced by collagen are known as what? alpha-chains
why are alpha-chains the opposite of the normal direction? high proportion of proline residues which contort the polypeptide backbone
most prolines follow what amino acid on the helix? lysine
where is collagen produced in the body? bones? cartilage? fibroblasts, osteoblasts, chondroblasts
after being processed in the cell, collagen is exported for completion in what form? tropocollagen
fibrilin mutation result in what disease? marfan's syndrome
what formations congregate to a scaffold of glycoprotein microfibrils such as fibrillin? tropoelastins
unlike collagen, tropoelastins are not bundled into parallel fibrils. Instead, they form what structures? crisscrossed meshworks
Tropoelastin consists of alternating what? hydrophobic and hydrophilic segments
what is an inhibitor of various proteases such as elastase, which is secreted by neutrophils? alpha-1-antitrypsin
alpha-1-AT-deficiency can lead to what disease? emphysema
what are polysaccharides composed of disaccharide repeats which are modified with nitride, sulfide or carboxyl groups? Glycosaminoglycans
an extremely large glycosaminoglycan, consisting of up to 50,000 glucuronic acid / acetylglucosamine repeats: Hyaluronan (hyaluronic acid)
The polar sugars of hyaluronan globs interact with water molecules to form gel like bodies known as what? hydration spheres
what is the function of hydration spheres? provide turgor to tissues, increase viscosity of extracellular fluids, and serve as lubricants for joints
glycosaminoglycan-protein compounds are known as what? proteoglycans
what is a proteoglycan that activates a clotting inhibitor called antithrombin III? heparin sulfate
these consist of as many as 100 proteoglycans linked to a hyaluronan core: aggrecans
what is the function of aggrecans? to resist compression
extracellular matrix crosslinking proteins are called what? multiadhesive matrix proteins
what structures crosslink the extracellular matrix and anchor it to cells? fibronectins
what structures crosslink extracellular matrix fibers forming the basal lamina? laminin
Created by: mhassan