#2 Pemphigus is a rare autoimmune disease that causes watery blisters to form al
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Question
#2 Pemphigus is a rare autoimmune disease that causes watery blisters to form all over the skin, including and especially the mucous membranes of the mouth and nose. These blisters are extremely itchy and easily pop or fall off, developing into deep sores that are prone to nasty infections. It was found that pemphigus is caused by the production of autoantibodies to a family of proteins called desmogleins, which is within the superfamily of proteins known as cadherins.
A.) What are cadherins and what structures do they help form?
B.) What cellular structures specifically have the desmoglein proteins and where are these found in the human body?
C.) Explain the symptoms of pemphigus, given the underlying cause.
D.) It turns out that pemphigus is actually a family of related conditions, with each version of the disease caused by autoimmune attack against a different member of the desmoglein family. Describe two specific types of pemphigus, which demiglein is being attacked, and why it has the specific features that it does
Explanation / Answer
A. Cadherins are calcium dependent class of type-1 transmembrane proteins. They form adherens junctions which bind the tissues together. Their primary role is cell adhesion.
B. All the cells will have desmoglein proteins and these structures are found in the cell to cell adhesion of epithelial cells.
C. Symptoms include epidermis being unglued called acantholysis, also blisters develop all over the skin.
D. Two types of pemphigus namely
Pemphigus Vulgaris: Antibodies attack the desmoglein 3 protein. Sores develop originate in the mouth.
Pemphigus Foliaceus: Antibodies attack the desmoglein 1 protein. Sores are found on the top layer of the skin only.
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