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16 Cards in this Set
- Front
- Back
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Main components of ECM
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collagens
elastic fibers proteoglycans hyaluronan glycoproteins |
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type I collagen
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Fibril forming
-skin, bone, tendon, blood vessels, cornea |
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type II collagen
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fibril forming
-Cartilage, vertebral disc, vitreous body |
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type III collagen
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fibril forming
-blood vessels, fetal skin |
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type IV collagen
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network forming
-basement membrane |
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basic structure of collagen
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-triple helix
-can be homo or hetero chains -produced as procollagen -primary structure is gly-x-y where x and y are typically proline and hydroxy proline |
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collagen fibrils
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formed by collagen monomers in a staggered formation.
stabilized by crosslinking |
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EDS V
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classic Ehlers Danlos
-hyper extensive skin -delayed wound healing -joint hypermobility mutation in type V collagen |
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EDS III
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Vascular Ehlers Danlos
-arterial, intestinal, and uterine(during pregnancy) ruptures mutation in type III collagen |
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kyphoscoliotic EDS
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Mutation in lysl hydroxylase
-hyper extensive skin -delayed wound healing -joint hypermobility -progressive scoliosis |
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Osteogenesis imprefecta
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types I,II,III,IV have type 1 collagen mutation
-Increases incidence of fractures -short stature -blue sclera -grey or brown teeth that wear down easily |
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Alport syndrome
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-type IV collagen mutation
-affects glomerular basement membrane -hematuria -proteinuria -renal insufficiency |
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elastin
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-produced in ER
-secreted as monomers -main location is blood vessels and lungs -correct assemnbly requres microfibrils formed by fibrillins -lysl oxidase cross links lysine residues |
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Alpha 1 antitrypsin deficiency
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-neutrophil elastase degrades elastin in lung
-Alpha 1 antitrypsin inhibits degradation -lack of this enzyme gives rise to emphysema because of collapse of alveoli upon exhalation -exacxerbated in smokers |
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marfan syndrome
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-deficiency in fibrillin-1
-very tall with kyphoscoliosis -disproportionate length of limbs, fingers,toes -hyperflexible joints -dislocation of lens of eye -dilation of aorta -heart valve problems |
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junctional epidermisbullosa
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deficiency in laminins/integrins
-affects basement membrane below epidermis and mucus membrane -causes extreme blistering |
