by Lund University A montage of three images of single striatal neurons transfected with a disease-associated version of huntingtin, the protein that causes Huntington’s disease. Nuclei of untransfected neurons are seen in the background (blue). The neuron in the center (yellow) contains an abnormal intracellular accumulation of huntingtin called an inclusion body (orange). Credit: Wikipedia/ Creative...
by University College London A montage of three images of single striatal neurons transfected with a disease-associated version of huntingtin, the protein that causes Huntington’s disease. Nuclei of untransfected neurons are seen in the background (blue). The neuron in the center (yellow) contains an abnormal intracellular accumulation of huntingtin called an inclusion body (orange). Credit: Wikipedia/...
IOS PRESS IMAGE: Proposed pathway of mutant huntingtin (mHtt) contribution to cognitive dysfunction and cell death through impairments in synaptic autophagy: the Huntingtin protein (mHtt) interferes with autophagic efficiency, leading to a decline in synaptic autophagy. This may in turn interfere with synaptic plasticity, causing both cognitive dysfunction and loss of normal synaptic input to...
UNIVERSITY OF CALIFORNIA – IRVINE IMAGE: SHOWN IS A SURFACE RENDERING OF G3BP1 GRANULES (IN GREEN) DETECTED BY IMMUNOFLUORESCENCE IN THE HD MOUSE MODEL CORTEX. CREDIT: UCI SCHOOL OF MEDICINE Irvine, CA – June 23, 2021 – A new University of California, Irvine-led study finds that the persistence of a marker of chronic cellular stress,...
PRILENIA Newly published papers further elucidate the mechanisms underlying pridopidine’s neuroprotective properties through activation of the Sigma-1 Receptor (S1R). Pridopidine enhances mitochondrial function and reduces mHTT-induced ER stress, which are impaired in HD, mediated by the S1R. Three new peer-reviewed publications highlight pridopidine’s therapeutic potential and provide data supporting the role of the S1R in...
by Università di Trento In Huntington’s Disease, axonal transport is defective and this leads to neuronal degeneration. In this work, we found that the protein mutated in Huntington’s Disease, called huntingtin (HTT), needs to be methylated by the enzyme PRMT6 in order to guarantee an efficient axonal transport and the survival of neural cells (in...
by The Scripps Research Institute Disease-causing huntingtin, shown in red, interacts with ribosomes, shown in green, in a striatal neuron. The nucleus is blue. Credit: Nicolai Urban of Max Planck Institute for Neuroscience in Jupiter, Florida. In 1993, scientists discovered that a single mutated gene, HTT, caused Huntington’s disease, raising high hopes for a quick cure. Yet today,...
by Massachusetts Institute of Technology Geomic created plots of the data that mapped differences pertaining to 4,300 genes along dimensions such as mouse age, the extent of Huntington’s-causing mutation, and cell type (certain neurons and astrocytes in a region of the brain called the striatum are especially vulnerable in Huntington’s). The plots took the form of...
Huntington’s disease is a fatal inherited disorder that strikes most often in middle age with mood disturbances, uncontrollable limb movements, and cognitive decline. Years before symptom onset, brain imaging shows degeneration of the striatum, a brain region important for the rapid selection of behavioral actions. As the striatal neurons degenerate, their “identity” proteins, the building...
Scientists at EPFL’s Brain Mind Institute have identified an enzyme that can play a central role in developing a new route of treatment for Huntington’s Disease. ECOLE POLYTECHNIQUE FÉDÉRALE DE LAUSANNE USING AN IN VITRO KINOME SCREEN, THE LAB OF HILAL LASHUEL AT EPFL IDENTIFIED A NOVEL KINASE (TBK1) THAT PHOSPHORYLATES THE HUNTINGTIN PROTEIN AT...
