Gene therapy to replace the faulty CLN2 gene, which causes a neurodegenerative disease that is fatal by age 8-12 years, was able to slow significantly the rate of neurologic decline in treated children, according to a paper published online ahead of print in the May 2008 issue (Vol. 19 No. 5) of Human Gene Therapy, a peer-reviewed journal published by Mary Ann Liebert, Inc.

Researchers from the University of Pennsylvania School of Medicine are developing a novel approach to screen for drugs to combat neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS), or Lou Gehrig's disease, using yeast cells. In recent months a number of mutations have been found in a disease protein called TDP-43, which is implicated in ALS and certain types of frontotemporal dementia (FTD).

Leaky blood vessels that lose their ability to protect the spinal cord from toxins may play a role in the development of amyotrophic lateral sclerosis, better known as ALS or Lou Gehrig’s disease, according to research published in the April issue of Nature Neuroscience.

Iron is vital to human life; for example, it is a component of hemoglobin, the substance that makes our blood red and supplies our cells with oxygen. However, iron can also cause heavy damage; it is thought that iron deposits in the brain contribute to certain forms of neurodegenerative diseases such as Parkinsons’s, Huntington’ s, and Alzheimer’s.

Prion infection of neurons increases the free cholesterol content in cell membranes. A new study published in the online open access journal BMC Biology suggests that disturbances in membrane cholesterol may be the mechanism by which prions cause neurodegeneration and could point to a role for cholesterol in other neurodegenerative diseases.

The amyloid plaques found in the brains of Alzheimer’s disease patients may form much more rapidly than previously expected.

Tauopathies are neurodegenerative diseases that involve dysfunction of the microtubule-associated protein Tau. For example, hyperphosphorylated Tau forms the paired helical filaments (PHFs) found in Alzheimer’s disease.

Working with fruit flies, researchers have discovered a new mechanism by which the abnormal protein in Huntington’s disease causes neurodegeneration.

Diseases that cause neurons to break-down, such as Alzheimer's, Multiple Sclerosis and Creutzfeldt-Jakob disease (Mad Cow Disease), continue to be elusive to scientists and resistant to treatments.

A study published in Nature Neuroscience demonstrates that the excess of glucose chains induces neuronal death and causes Lafora disease, a fatal kind of epilepsy that affects adolescents

Researchers report the most complete list so far of the proteins present in the cerebral cortex – the outermost layer of the brain that plays a central role in memory, language, cognition, and consciousness.

The evidence is accumulating on how bad stress is for health. Chronic stress can intensify inflammation and increase a person’s risk for developing central nervous system infections, neurodegenerative diseases, like multiple sclerosis (MS), and other inflammatory diseases, say researchers presenting at the 115th Annual Convention of the American Psychological Association (APA).