Research in Maori Community Yields Hope for Huntington’s

I have just returned from a couple weeks in New Zealand, where I am collaborating with Dr. Melanie Cheung and others on a pretty incredible Huntington’s disease project. I am so inspired by what we’re doing there that I wanted to share a bit about it. More than 7,000 of the 4.5 million citizens of […]The post Research in Maori Community Yields Hope for Huntington’s appeared first on "On the Brain" with Dr. Michael Merzenich.
Source: On the Brain by Dr. Michael Merzenich, Ph.D. - Category: Neuroscience Authors: Tags: Brain Plasticity Brain Science Cognitive impairments Neuroscience Posit Science HD huntington's maori melanie cheung new zealand Source Type: blogs

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Molecules, Vol. 25, Pages 482: Huntington’s Disease: A Review of the Known PET Imaging Biomarkers and Targeting Radiotracers Molecules doi: 10.3390/molecules25030482 Authors: Klaudia Cybulska Lars Perk Jan Booij Peter Laverman Mark Rijpkema Huntington’s disease (HD) is a fatal neurodegenerative disease caused by a CAG expansion mutation in the huntingtin gene. As a result, intranuclear inclusions of mutant huntingtin protein are formed, which damage striatal medium spiny neurons (MSNs). A review of Positron Emission Tomography (PET) studies relating to HD was performed, including clinical an...
Source: Molecules - Category: Chemistry Authors: Tags: Review Source Type: research
(Duke University) A Duke University research team has identified a new function of a gene called huntingtin, a mutation of which underlies the progressive neurodegenerative disorder known as Huntington's Disease. Using genetic mouse models, they have discovered that neurons in the striatum, a brain area involved in controlling movement, require the huntingtin gene for regulating the body's movements, maintaining cell health during aging, and developing functioning connections between cells.
Source: EurekAlert! - Medicine and Health - Category: International Medicine & Public Health Source Type: news
Myelin is the sheathing of nerves, essential to their function. Excessive loss produces disabling and ultimately fatal conditions such as multiple sclerosis, but we all lose myelin integrity to some degree as a consequence of the damage and dysfunction of degenerative aging. This most likely contributes to cognitive decline and other age-related issues. A number of different approaches have been identified to boost the operation of the normal maintainance processes that remyelinate nerves, such as FGF21 upregulation, or increasing the size of remyelinating cell populations. Here, researchers discover another possible trigg...
Source: Fight Aging! - Category: Research Authors: Tags: Daily News Source Type: blogs
ino A Abstract Neurodegenerative diseases affect millions of people around the world. Several studies point out caspase-3 as a key player in the development and progression of neurological disorders including amyotrophic lateral sclerosis, Alzheimer's, Parkinson's and Huntington's diseases. Furthermore, oxidative stress and mitochondrial dysfunction plays an important role in neurodegenerative pathologies leading to neuronal damage and cell death. Pharmacological properties of nitrones such as free radical trapping and neuroprotection has been previously described. In the present work, we have assessed ten non-cyt...
Source: European Journal of Pharmacology - Category: Drugs & Pharmacology Authors: Tags: Eur J Pharmacol Source Type: research
Huntington's disease-like 2 (HDL-2) is an autosomal dominant neurodegenerative disorder caused by a CTG/CAG trinucleotide repeat expansion ( ≥40 repeats) in the junctophilin-3 gene on chromosome 16q24.3 [1]. The affected protein product, junctophilin-3, plays a role in anchoring the endoplasmic reticulum to the plasma membrane. The disorder generally manifests in mid-life as a Huntington's disease (HD) phenocopy syndrome comprising cog nitive decline, neuropsychiatric manifestations and movement disorders (generally chorea) [2,3].
Source: Parkinsonism and Related Disorders - Category: Neurology Authors: Tags: Correspondence Source Type: research
Abstract RNautophagy and DNautophagy (RDA) are unconventional autophagic pathways where nucleic acids are directly transported through the lysosomal membrane, then degraded inside lysosomes. We have previously shown that bitopic protein LAMP2C and putative RNA transporter SIDT2, both lysosomal membrane proteins, mediate the direct transport of nucleic acids into lysosomes and that LAMP2C interacts with the nucleic acids and functions as a receptor during RDA. Because SIDT2-mediated RDA occurs in isolated lysosomes that lack LAMP2C, in this study, we tested the hypothesis that SIDT2 itself could also interact with ...
Source: Autophagy - Category: Cytology Authors: Tags: Autophagy Source Type: research
In the original version of the paper, the name of one of the contributing authors, Dr. Mundackal S. Divya (orcid:0000-0002-2869-7191).
Source: Molecular Neurobiology - Category: Neurology Source Type: research
Abstract The global burden of neurodegenerative diseases is alarmingly increasing in parallel to the aging of population. Although the molecular mechanisms leading to neurodegeneration are not completely understood, excitotoxicity, defined as to the injury and death of neurons due to an excessive or prolonged exposure to excitatory amino acids, has been shown to play a pivotal role. The increased release and/or decreased uptake of glutamate results in a dysregulation of neuronal calcium homeostasis, leading to oxidative stress, mitochondrial dysfunctions, disturbances in protein turn-over and neuroinflammation. De...
Source: Current Pharmaceutical Design - Category: Drugs & Pharmacology Authors: Tags: Curr Pharm Des Source Type: research
AbstractHuntington ’s disease is a dominantly inherited neurodegenerative disease caused by an unstable expanded trinucleotide repeat at the short end of the fourth chromosome. Central nervous system pathology begins in the striatum, eventually affecting the entire brain and occurs consequent to multiple intracellul ar derangements. The proximate cause is a mutant protein with an elongated polyglutamine tract. Pharmacological approaches targeting multiple domains of intracellular functions have universally been disappointing. However, recent developments in gene therapy, including antisense oligonucleotides, sm all i...
Source: CNS Drugs - Category: Neurology Source Type: research
Abstract Mitochondria-associated membranes (MAMs) are dynamic structures that communicate endoplasmic reticulum (ER) and mitochondria allowing calcium transfer between these two organelles. Since calcium dysregulation is an important hallmark of several neurodegenerative diseases, disruption of MAMs has been speculated to contribute to pathological features associated with these neurodegenerative processes. In Huntington's disease (HD), mutant huntingtin induces the selective loss of medium spiny neurons within the striatum. The cause of this specific susceptibility remain unclear. However, defects on mitochondria...
Source: Neurobiology of Disease - Category: Neurology Authors: Tags: Neurobiol Dis Source Type: research
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