Cholesterol and HD

An investigation into how HD affects cholesterol homeostasis

Implications

Cholesterol is essential for promoting synapse formation and maintaining membrane integrity in CNS neurons. It is also a major component of myelin and important for optimal neurotransmitter release. Because cholesterol plays such a major role in CNS growth, development, and maintenance, disruptions of cholesterol homeostasis can have negative consequences. Accumulation and depletion of intracellular cholesterol in neurons are both possible mechanisms contributing to neuron dysfunction in these HD models. However, the findings are limited to HD cell models and postmortem HD tissue. This work now needs to be followed up by investigating these changes in HD patients to see whether similar dysfunction occurs.

If studies in human subjects found a similar dysfunction in cholesterol homeostasis, it might suggest that adjusting the cholesterol levels in neuronal cells could be a potential treatment for HD. Future research may aim to discover how to transport cholesterol across the blood brain barrier and whether cholesterol therapy could be one way of slowing or halting neuronal cell death in HD.

It is interesting to note that similar defects in caveolar-related endocytotic pathways and perturbations of cholesterol homeostasis have been implicated in other neurodegenerative diseases related to HD like Alzheimer's disease and Parkinson's disease.

Last Modified: 07/07/2007

An educational product of HOPES, not to be used in place of medical care. For more information about HOPES, click on the Logo. To contact HOPES with comments or questions, click here.