Huntington’s Disease: A Neurodegenerative Disorder
摘要
Huntington’s disease is a rare, progressive, and deadly hero-neurodegenerative illness that causes cognitive, behavioral, and motor impairment. The condition is autosomal dominant, meaning that all it takes to pass it down is one copy of the defective gene. The Huntington (HTT) gene, which codes for the Huntingtin protein, has undergone a mutation that results in an excess of CAG repeats close to its 5′ end [1]. The mutation causes the poly-glutamine in the HTT protein to expand excessively long, which causes neurodegeneration. Those between the ages of 35 and 50 are the group that is most affected. The striatal portion of the ganglia exhibits selective atrophy and cell death as a result of this adult-onset disease [2]. Although the range for those affected is thought to be 36–120 triplet repeats, persons with more than 35 triplet repeats are more likely to have this illness. Most people who have fewer than 35 triplet repeats are unaffected.