**Core Concept**
Kinky hair disease, also known as Menkes disease, is a rare disorder caused by a mutation in the ATP7A gene. This gene is responsible for encoding a copper-transporting ATPase, which plays a crucial role in copper distribution and utilization in the body. The disorder is characterized by progressive brain degeneration, sparse and brittle hair, and early death.

**Why the Correct Answer is Right**
Menkes disease is an X-linked recessive disorder, meaning it is caused by mutations in genes located on the X chromosome. In this case, Mrs. A's two sisters had sons with the disease, indicating that the sisters are carriers of the mutated gene. The disease manifests in males, who have only one X chromosome and therefore lack a functional copy of the ATP7A gene. The fact that Mrs. A's mother's brother also died of the same condition suggests that the disorder is inherited in an X-linked recessive pattern. This pattern of inheritance is characterized by affected males and carrier females, who may pass the mutated gene to their sons.

**Why Each Wrong Option is Incorrect**
**Option A:** Autosomal dominant inheritance would not explain the absence of affected females in the family.
**Option B:** Autosomal recessive inheritance would not explain the sex-specific manifestation of the disease in males.
**Option C:** Mitochondrial inheritance would not explain the involvement of the X chromosome and the absence of affected females.
**Option D:** X-linked dominant inheritance would not explain the absence of affected females in the family and the severity of the disease in males.

**Clinical Pearl / High-Yield Fact**
Menkes disease is a classic example of an X-linked recessive disorder, where males are affected and females are carriers. This pattern of inheritance is essential to recognize in clinical practice, as it can help identify affected individuals and carriers within families.

**Correct Answer:** . X-linked recessive.