18. Huntington disease is _____.
a model of a multifactorial trait, controlled by several genes
inherited as an X-linked dominant trait
a disease with onset in early childhood
one that produces no significant changes in brain cells
a model of a single gene defect affecting behavior
18–5: Huntington Disease Is a Model for Neurodegenerative Disorders
19. Why is the most damage to the brains of Huntington disease patients observed in the striatum?
The Rhes protein, needed for mutant huntingtin to become toxic, is only produced in the striatum.
The huntingtin gene is only expressed in the striatum.
The mutant huntingtin protein becomes toxic when bound to the Rhes protein, which is abundant in the
striatum.
Only the cells of the striatum contain the huntingtin mutation.
The CAG expansions are corrected in regions of the brain other than the striatum.
18–5: Huntington Disease Is a Model for Neurodegenerative Disorders
20. Research on transgenic Drosophila models of Huntington disease has found that ____.
the NUB1 protein decreases levels of mutant huntingtin protein, and thereby, cell death
the mutant huntingtin protein is destroyed by the SOD1 protein
Drosophila and humans share more than 90% of genes
the accumulation of mutant proteins in the brain causes Huntington disease
treatment of patients with Rhes protein cures Huntington disease
18–6: Animal Models: The Search for Behavior Genes
21. Research using transgenic animal models of neurodegenerative diseases not only helps scientists understand the
disorders, but also may lead to _____.
Drosophila genome sequencing projects
discovery of the environmental causes of schizophrenia
identification of the mutant gene that causes Huntington disease
directly related to abnormal behavior in one family.