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APS (Autoimmune Polyendocrine Syndrome) Type 1

Answer :

Final answer:

APS Type 1, or APECED, is a rare inherited disorder where the immune system incorrectly attacks healthy organs and tissues. This primarily affects the endocrine glands and is caused by mutations in the AIRE gene.

Explanation:

APS (Autoimmune polyendocrine syndrome) Type 1, also known as APECED (Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy), is a rare inherited disorder characterized by the abnormal functioning of the body's immune system. This leads to the immune system mistakenly attacking healthy organs and tissues, specifically endocrine glands such as the parathyroids and the adrenal glands. As a result, affected individuals may experience multiple endocrine deficiencies, chronic mucocutaneous candidiasis, and various ectodermal abnormalities (e.g., dental enamel hypoplasia). The syndrome is caused by mutations in the AIRE (AutoImmune REgulator) gene, which is involved in immune system regulation.

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