“Menke’s disease” is a disease of-
**Core Concept**
Menke's disease is a rare, inherited disorder characterized by impaired copper metabolism, resulting in copper deficiency and accumulation of toxic copper in the body. It is an example of a genetic disorder affecting the **ATP7A gene**, responsible for encoding a copper-transporting ATPase involved in copper absorption and distribution.
**Why the Correct Answer is Right**
Menke's disease primarily affects the **brain**, **spinal cord**, and **peripheral nerves**, leading to symptoms such as seizures, developmental delays, and peripheral neuropathy. The impaired copper metabolism also affects the **skin**, causing hair loss, brittle hair, and skin lesions. The defective ATP7A protein fails to transport copper into the **lysosome**, where it is needed for the production of **ceruloplasmin**, a copper-containing protein that plays a crucial role in copper metabolism.
**Why Each Wrong Option is Incorrect**
**Option A:** Menke's disease is not primarily a disorder of the **pancreas**; it does not result in diabetes mellitus or pancreatic insufficiency.
**Option B:** Although Menke's disease affects the **nervous system**, it is not a demyelinating disorder like **Multiple Sclerosis**.
**Option C:** Menke's disease is not a disorder of **iron metabolism**; it is specifically related to copper metabolism.
**Clinical Pearl / High-Yield Fact**
Menke's disease is an X-linked recessive disorder, predominantly affecting males. The disease is characterized by a characteristic "kinky" or "steely" hair, which is a key diagnostic feature.
**Correct Answer: C. Peripheral nerves.**