Hope for Huntington's Disease Patients

Ian C. Langtree - Writer/Editor for Disabled World (DW)
Published: 2016/01/24 - Updated: 2024/12/25
Publication Type: Findings
Topic: Medical Research News - Publications List

Page Content: Synopsis - Introduction - Main

Synopsis: Texas Tech University Health Sciences Center researchers identify promising molecule for treatment of Huntington's Disease. Huntington's disease is a genetic disease that causes the progressive destruction of striatal neurons in the brain. According to the National Institute of Neurological Disorders and Stroke (NINDS), Huntington's disease is known as a family disease because every child of a parent with the disease has a 50 percent chance of inheriting the disease.

Introduction

An estimated 30,000 Americans have Huntington's disease and more than 200,000 people are at risk of inheriting it. Texas Tech University Health Sciences Center (TTUHSC) researchers identified a mitochondrial division inhibitor 1 (Mdivi1) that may be a promising molecule for the treatment of patients with this disease.

Main Item

P. Hemachandra Reddy, Ph.D., executive director and chief scientific officer of the TTUHSC Garrison Institute on Aging and School of Medicine professor in the departments of Cell Biology & Biochemistry, Neuroscience/Pharmacology and Neurology, and Maria Manczak, Ph.D., senior research associate at the Garrison Institute on Aging, led the research study, "Mitochondrial division inhibitor 1 protects against mutant huntingtin-induced abnormal mitochondrial dynamics, synaptic dysfunction and neuronal damage in Huntington's disease." The research article appeared in Human Molecular Genetics.

Mitochondria are specialized organelles in a cell and are responsible for creating energy needed by the body to sustain life.

"When the mitochondria are compromised in neurons from brain disease such as Huntington's, injury and even death to the cell occurs," Reddy said. "Mitochondrial damage and synaptic dysfunction are the prominent features in neurons from patients with Huntington's disease."

According to Reddy's research, mitochondrial division inhibitor 1 is capable of reducing mutant-huntingtin induced excessive mitochondrial fragmentation and maintaining mitochondrial function and improving synaptic activity in Huntington's disease neurons.

"These findings suggest that mitochondrial division inhibitor 1 will improve mitochondrial function and protect against synaptic damage in Huntington's disease," Reddy said. "This may be a promising molecule for treatment for the Huntington's disease patient."

Findings from this study may also have therapeutic implications to all brain diseases with expanded polyglutamine repeats as genetic mutation(s).

"Dr. Reddy's study findings are exciting and give hope to families struggling with Huntington's disease," said Michael Conn, Ph.D., senior vice president for Research and TTUHSC associate provost.

Huntington's disease is a genetic disease that causes the progressive destruction of striatal neurons in the brain. According to the National Institute of Neurological Disorders and Stroke, Huntington's disease is known as a family disease because every child of a parent with the disease has a 50 percent chance of inheriting the disease.

Genetic mutation that causes disease is due to the presence of expanded (more than 35) polyglutamine or CAG repeats in Huntington's disease gene. Mutant huntingtin is a protein that expresses expanded polyglutamine repeats in all cells from patients with Huntington's disease, but selectively targets striatal neurons in the basal ganglia.

Reddy said symptoms of Huntington's disease can include progressive deterioration of cognitive processes and memory, uncontrolled, irregular, rapid, jerky movements called chorea and athetosis, a condition characterized by relatively slow involuntary movements.

Author Credentials: Ian was born and grew up in Australia. Since then, he has traveled and lived in numerous locations and currently resides in Montreal, Canada. Ian is the founder, a writer, and editor in chief for Disabled World. Ian believes in the Social Model of Disability, a belief developed by disabled people in the 1970s. The social model changes the focus away from people's impairments and towards removing barriers that disabled people face daily. To learn more about Ian's background, expertise, and achievements, check out his bio.