Posters

Presenting Author

Elena Dike

Presenting Author Academic/Professional Position

Medical Student

Academic Level (Author 1)

Medical Student

Academic Level (Author 2)

Medical Student

Academic Level (Author 3)

Faculty

Discipline/Specialty (Author 3)

Internal Medicine

Presentation Type

Poster

Discipline Track

Clinical Science

Abstract Type

Research/Clinical

Abstract

Background: Huntington’s disease (HD) is a progressive neurodegenerative disorder caused by a CAG trinucleotide repeat expansion in the HTT gene. While CAG repeat length is the primary determinant of disease onset, substantial variability exists in the rate of disease progression and cognitive decline. This variability is influenced by both cis and trans genetic modifiers, including DNA repair genes such as MLH1, MSH3, PMS2, and FAN1. Emerging evidence also suggests that systemic metabolic comorbidities—including diabetes mellitus, dyslipidemia and hypertension—may exacerbate neuronal vulnerability and accelerate neurodegeneration.

Methods: We propose a retrospective, longitudinal cohort study utilizing existing HD datasets containing genetic, metabolic, and cognitive data. Genetic variables of interest will include HTT CAG repeat length and known DNA repair modifier genes (MLH1, MSH3, PMS2, FAN1). Metabolic factors will include the presence of diabetes mellitus, dyslipidemia, and hypertension. Cognitive outcomes will be assessed using standardized neuropsychological measures collected longitudinally. Planned analyses include multivariable regression modeling and interaction analyses to evaluate whether metabolic comorbidities modify the relationship between genetic risk factors and cognitive decline over time.

Results: We hypothesize that the presence of metabolic comorbidities will be associated with accelerated cognitive decline and that these effects will be amplified in individuals carrying high-risk genetic modifier profiles.

Conclusion: This protocol highlights a novel integrative framework examining both genetic and metabolic contributors to disease progression in Huntington’s disease. Identifying modifiable metabolic factors that interact with genetic risk may inform earlier intervention strategies and improve long-term quality of life for individuals living with HD.

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Proposed Protocol to Evaluate the Interaction of Genetic Modifiers and Metabolic Comorbidities on Cognitive Decline in Huntington’s Disease

Background: Huntington’s disease (HD) is a progressive neurodegenerative disorder caused by a CAG trinucleotide repeat expansion in the HTT gene. While CAG repeat length is the primary determinant of disease onset, substantial variability exists in the rate of disease progression and cognitive decline. This variability is influenced by both cis and trans genetic modifiers, including DNA repair genes such as MLH1, MSH3, PMS2, and FAN1. Emerging evidence also suggests that systemic metabolic comorbidities—including diabetes mellitus, dyslipidemia and hypertension—may exacerbate neuronal vulnerability and accelerate neurodegeneration.

Methods: We propose a retrospective, longitudinal cohort study utilizing existing HD datasets containing genetic, metabolic, and cognitive data. Genetic variables of interest will include HTT CAG repeat length and known DNA repair modifier genes (MLH1, MSH3, PMS2, FAN1). Metabolic factors will include the presence of diabetes mellitus, dyslipidemia, and hypertension. Cognitive outcomes will be assessed using standardized neuropsychological measures collected longitudinally. Planned analyses include multivariable regression modeling and interaction analyses to evaluate whether metabolic comorbidities modify the relationship between genetic risk factors and cognitive decline over time.

Results: We hypothesize that the presence of metabolic comorbidities will be associated with accelerated cognitive decline and that these effects will be amplified in individuals carrying high-risk genetic modifier profiles.

Conclusion: This protocol highlights a novel integrative framework examining both genetic and metabolic contributors to disease progression in Huntington’s disease. Identifying modifiable metabolic factors that interact with genetic risk may inform earlier intervention strategies and improve long-term quality of life for individuals living with HD.

 

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