If cellular proteins do not fold into a specific conformation, their function is affected. Ceain disorders arise if specific proteins are misfolded. Which of the following disorders arises due to conformational isomerization?
**Core Concept**
Protein folding is a crucial process where polypeptide chains arrange themselves into specific three-dimensional structures. These structures determine the function and stability of proteins. Misfolding of proteins can lead to various disorders due to altered function or toxicity.
**Why the Correct Answer is Right**
Conformational isomerization refers to the change in the three-dimensional structure of a protein. This can occur due to mutations, stress, or other factors. One such disorder is Creutzfeldt-Jakob disease, a neurodegenerative disorder caused by the misfolding of the prion protein. Prions are infectious proteins that can induce normal proteins to misfold, leading to cell death and neurodegeneration.
**Why Each Wrong Option is Incorrect**
**Option A:** Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disorder, but it is primarily caused by the accumulation of misfolded superoxide dismutase 1 (SOD1) protein, not conformational isomerization.
**Option B:** Huntington's disease is an autosomal dominant disorder caused by an expansion of CAG repeats in the Huntingtin gene, leading to a toxic protein fragment. While protein misfolding is involved, it is not due to conformational isomerization.
**Option C:** Alzheimer's disease is a complex disorder involving the accumulation of amyloid beta plaques and tau tangles. While protein misfolding is a key feature, the primary cause is not conformational isomerization.
**Clinical Pearl / High-Yield Fact**
Creutzfeldt-Jakob disease is a rare, fatal neurodegenerative disorder that highlights the importance of protein folding and misfolding in disease pathogenesis. This condition also underscores the potential for infectious proteins to cause disease.
**Correct Answer:** C. Creutzfeldt-Jakob disease.