Alport Syndrome
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Alport syndrome is an X-linked dominant disorder that is characterized by a mutation in type IV collagen. As a result of this defect, patients with Alport syndrome have defective basement membranes in various organs. Defects in the glomerular basement membrane lead to glomerulonephritis, which manifests as a "basket-weave" pattern on electron microscopy. Alport syndrome patients also develop a number of eye problems, as basement membranes are found in the lens capsule and retina. Such eye problems include cataracts, retinopathy, and lens dislocation. Finally, defects in the cochlear basement membrane lead to sensorineural deafness in patients with Alport syndrome.