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10 Cards in this Set

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  • Back
collagen
trimeric protein
3 polypeptide chains forming a triple helical structure
fibrillar: in connective tissue: insoluble, crosslinked fibers of high tensile strength
n---have a banded appearance
nonfibrillar: bm, assemble to form networks rather than fibers
collagen
trimeric protein
3 polypeptide chains forming a triple helical structure
fibrillar: in connective tissue: insoluble, crosslinked fibers of high tensile strength
n---have a banded appearance
nonfibrillar: bm, assemble to form networks rather than fibers
fibronection
large, disulfid-linked, dimeric protein secreted by and found on teh surface of many cell types. . such as fibroblasts, myoblasts and basal surface of many epitheliaal cells. can also provide a surface on which cells can migrate...distinct domains bind distinct coponents.
single gene encodes fn but several different forms arise due to alternative splicing.
elastin
major protein that forms elastic fibers
-prom in blood vessesl, lungs and skin
-cores is covered by a sheath of microfibrils, composed of several proteins----one is fibrillin which binds to elastin ans is critical for the assembly of elastic fibers
marfan's syndrome
results from mutations in fibrillin
-tall with long fingers, arms and legs.
-walls of major blood vessels are often weak and heart is enlarged.
glycosaminoglycans (GAGS) and proteoglycans
GAGS: long linear chains of repeating disaccharide units (one acidic sugar and one amino sugar). highly negatively charged adn frequently sulfated.---
*are covalently linked to proteins, making proteoglycans.
bc highly charged adn hydrated, they occupy large volumes. tend to resist compressive forces, important for generating cavities, especially in embryos.
proteoglcans
core porteins covalently linked to GAGS. prominent compentent sof ECM and bind to mahy of the other compoentns.
integrins
large family of membrane proteins that serve as receptors for many ECM proteins. heterodimers, consisting of noncovalently associated alpha and b domains.
-both polypep span the plasma membrane and have short cytoplasmi comains...defined by b chain. each b chain can complex with a number of difernt alpha chains, generating receptors with different specificities
leukocyte adhesion defiiciency
in which individuals do not express the b2 family of integrins, as a result many leukocytes are unable to adhere properly ot the endothelium in response to inflammation and cannot migrate into sites of infection and inflammation. (don't express the b2 family of integrins)
glanzmann's thrombasthenia
these individuals lack the major integrin on blood platelets, normallyu involved in bidning to fibrinogen/ fibrin during blood clotting. ..hemophelia