Huntington’s disease is due to the loss of :
**Core Concept**
Huntington's disease (HD) is a rare, autosomal dominant neurodegenerative disorder characterized by progressive damage to the brain, leading to motor dysfunction, cognitive decline, and psychiatric symptoms. The disease is caused by an expansion of a CAG repeat in the Huntingtin gene, resulting in a toxic protein that disrupts normal cellular function.
**Why the Correct Answer is Right**
The correct answer is associated with the pathogenesis of Huntington's disease. The Huntingtin protein contains a polyglutamine tract that is normally short, but in HD, the CAG repeat expansion leads to an elongation of this tract. This expansion causes the protein to misfold and aggregate, forming toxic inclusions that disrupt normal cellular function, particularly in the basal ganglia and cortex. The expanded polyglutamine tract also leads to the activation of various cellular pathways, including the ubiquitin-proteasome system and the autophagy-lysosome pathway, which ultimately contribute to neuronal death.
**Why Each Wrong Option is Incorrect**
**Option A:** This option is incorrect because it does not accurately describe the pathogenesis of Huntington's disease. While mitochondrial dysfunction has been implicated in various neurodegenerative disorders, it is not the primary cause of HD.
**Option B:** This option is incorrect because it is not directly related to the pathogenesis of Huntington's disease. While the striatum is a brain region that is affected in HD, the disease is not caused by the loss of this region.
**Option C:** This option is incorrect because it is too broad and does not specifically describe the pathogenesis of Huntington's disease. While oxidative stress and excitotoxicity are mechanisms that contribute to neuronal death in various neurodegenerative disorders, they are not the primary causes of HD.
**Clinical Pearl / High-Yield Fact**
Huntington's disease is an autosomal dominant disorder, meaning that a single copy of the mutated gene is sufficient to cause the disease. This has significant implications for genetic counseling and testing, as individuals with a family history of HD should undergo genetic testing to determine their risk of developing the disease.
**Correct Answer:** Not provided