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16 Cards in this Set
- Front
- Back
cells that make extracellular proteins
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fibroblasts
hepatocytes plasma cells |
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4 signs of osteogenesis imerfecta
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blue sclerae, multiple fractures, haring loss, dental imperefctions
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collagen defect in OI?
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type I
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symptoms of Ehlers Danlos
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hyperextensible skin
tendency to bleed hypermobile joints |
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collagen defect in ehlers-danlos?
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type III
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hydroxylation of collagen requires?
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citamin C
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translation of collagen alpha chains makes
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preprocollagen
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glycosylation of preprocollagen makes
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procollagen
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cleavage of terminial regions of procollagen makes
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tropocollagen
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collagen fibrils
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reinforced collagen- staggered tropocollagen molecules are covalently bonded by lysine-hydroxylase cross-linkage
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progressive heredtiary nephritis, deafness
may be associated with ocular disturbances |
Alport syndrome
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symptoms of Alport syndrome
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progressive hereditary nephritis
deafness possible ocular disturbance |
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results in emphysema due to unchecked elastase activity
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alpha 1 antitrypsin deficiency
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stretchy protein within lungs, large arteries, elastic ligaments, vocal cords, ligamenta flava (connect vertebrae- relaxed and stretched conformations)
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elastin
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enzyme that breaks down elastin
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elastase~
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what type of collagen is defective in Alport syndrome?
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IV
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