Researchers discover a three molecule complex could be a target for treating Huntington’s Disease, a genetic and currently incurable brain disease which causes movement disorders and dementia.
Voluntary movements involve the coordinated activation of two brain pathways that connect parts of deep brain structures called the basal ganglia, according to a study in mice.
Researchers identified 21 proteins that specifically bind to a protein called ataxin-1. Twelve of these proteins enhance the misfolding of ataxin-1 and thus promote the formation of harmful protein aggregate structures, whereas nine of them prevent the misfolding.
A new light-based technique for measuring levels of the toxic protein that causes Huntington’s disease (HD) has been used to demonstrate that the protein builds up gradually in blood cells.
HD mice crossbred with mice that produced greater levels of PGC-1alpha showed dramatic improvement. Production of misfolded proteins was essentially eliminated and the mice behaved normally. “Degeneration of brain cells is prevented. Neurons don’t die,” said La Spada.
‘HD in a dish’ will facilitate search for elusive treatment. An international consortium of Huntington’s disease experts, including several from the Sue and Bill Gross Stem Cell Research Center at UC Irvine, has generated a human model of the deadly inherited disorder directly from the skin cells of affected patients. The re-created neurons, which live [...]
Nerve Pathway for Combating Axon Injury and Stress May Hold Benefits for Individuals With Neurodegenerative Disorders
Researchers from the Huck Institutes’ Center for Cellular Dynamics, led by Center director Melissa Rolls, have found that a neuroprotective pathway initiated in response to injured or stressed neural axons serves to stabilize and protect the nerve cell against further degeneration. Neurons, or nerve cells, typically have a single axon that transmits signals to other [...]
Single treatment produces long-term improvement in animal models. With a single drug treatment, researchers at the Ludwig Institute for Cancer Research at the University of California, San Diego School of Medicine can silence the mutated gene responsible for Huntington’s disease, slowing and partially reversing progression of the fatal neurodegenerative disorder in animal models. The findings [...]
Findings could have broad therapeutic potential for many neurodegenerative diseases. Degeneration of the axon and synapse, the slender projection through which neurons transmit electrical impulses to neighboring cells, is a hallmark of some of the most crippling neurodegenerative and brain diseases such as amyotrophic lateral sclerosis (ALS), Huntington’s disease and peripheral neuropathy. Scientists have worked [...]
Perhaps no other biochemist in the world has his own baseball card, but University of Massachusetts Amherst doctoral student Elih M. Velazquez-Delgado, who gave up a pitching career for science, does. Now the only stats he cares about are experimental data, because, he says, “I fell in love with the fact that I can see [...]