MICROANATOMY

MUSCLE NERVE CARTILAGE BONE

GANGLIA

Question [CLICK ON ANY CHOICE TO KNOW THE RIGHT ANSWER]
In Huntington’s disease, the defect is in the ____
A
Subthalamic nucleus
B
Substantia nigra
C
Striatum
D
Lentiform nucleus
Explanation: 

Detailed explanation-1: -Huntington’s disease (HD) is a hereditary neurodegenerative disorder that typically manifests in midlife with motor, cognitive, and/or psychiatric symptoms. The disease is caused by a CAG triplet expansion in exon 1 of the huntingtin gene and leads to a severe neurodegeneration in the striatum and cortex.

Detailed explanation-2: -In patients with Huntington’s disease, neurons in a part of the brain called the striatum are among the hardest-hit. Degeneration of these neurons contributes to patients’ loss of motor control, which is one of the major hallmarks of the disease.

Detailed explanation-3: -The striatum is the input module to the basal ganglia, a neuronal circuit necessary for voluntary movement control (Hikosaka et al., 2000). The striatum is composed of three nuclei: caudate, putamen, and ventral striatum. The latter contains the nucleus accumbens (NAcc).

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