Alport's Syndrome is linked with a defect in which type of collagen?

Prepare for USMLE Step 1 Pathology Exam with comprehensive quizzes, flashcards, and detailed explanations. Enhance your understanding and be exam-ready!

Alport's Syndrome is primarily associated with a defect in Type IV collagen. This condition is a hereditary nephritis characterized by progressive renal failure, sensorineural hearing loss, and ocular abnormalities, which are primarily due to mutations affecting the collagen type responsible for the structure of the glomerular basement membrane in the kidneys.

Type IV collagen is particularly crucial in forming the basal lamina, including the glomerular membrane. In Alport's Syndrome, the dysfunction leads to abnormalities in the kidney's filtration barrier, which ultimately results in hematuria and proteinuria that can progress to chronic kidney disease.

Given the significance of Type IV collagen in the pathology of Alport's Syndrome, identifying it as linked to the condition aids in understanding the molecular basis of the disease and its clinical manifestations. Other types of collagen mentioned play roles in different structural components and tissues but are not directly involved in the pathogenesis of Alport's Syndrome.

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