The sisters with childhood Alzheimer's

Both Abby, three, and Belle, seven, have Niemann-Pick Type C1, a genetic disease where in time, children slowly regress until they can ’t walk, eat, and dementia will set in before age 10.
Source: the Mail online | Health - Category: Consumer Health News Source Type: news

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In this study, 7 – 8-month-old male TgAPP21 rats were tested for behavioral flexibility, learning, and memory using an operant conditioning chamber and the Morris Water Maze (MWM). TgAPP21 rats demonstrated a regressive behavioral inflexibility during set shifting in an operant conditioning chamber (regressive errors η2 = 0.32 and number of errors after criterion η2 = 0.33). Regressive behavior was also demonstrated in the MWM probe test, wherein TgAPP21 rats significantly increased their swim time in the target quadrant during the last third of the probe test (43% vs 33% in the first 2 thirds of the probe te...
Source: Brain, Behavior, and Immunity - Category: Neurology Source Type: research
DiscussionDuration of each preclinical stage can be estimated in ADAD, facilitating better planning of prevention trials with the EYO cutoffs under the recently released FDA guidance. The generalization of these results to sporadic AD warrants further investigation.
Source: Alzheimer's and Dementia: The Journal of the Alzheimer's Association - Category: Geriatrics Source Type: research
Publication date: Available online 14 February 2019Source: Stem Cell ReportsAuthor(s): Lydia Reinhardt, Susanne Kordes, Peter Reinhardt, Michael Glatza, Matthias Baumann, Hannes C.A. Drexler, Sascha Menninger, Gunther Zischinsky, Jan Eickhoff, Claudia Fröb, Prabesh Bhattarai, Guruchandar Arulmozhivarman, Lara Marrone, Antje Janosch, Kenjiro Adachi, Martin Stehling, Eric N. Anderson, Masin Abo-Rady, Marc Bickle, Udai Bhan PandeySummaryNeuroinflammation is a hallmark of neurological disorders and is accompanied by the production of neurotoxic agents such as nitric oxide. We used stem cell-based phenotypic screening and ...
Source: Stem Cell Reports - Category: Stem Cells Source Type: research
Publication date: Available online 7 February 2019Source: NeuroImageAuthor(s): Nan-Jie Gong, Russell Dibb, Marjolein Bulk, Louise van der Weerd, Chunlei LiuAbstractBeta amyloid is a protein fragment snipped from the amyloid precursor protein (APP). Aggregation of these peptides into amyloid plaques is one of the hallmarks of Alzheimer's disease. MR imaging of beta amyloid plaques has been attempted using various techniques, notably with T2* contrast. The non-invasive detectability of beta amyloid plaques in MR images has so far been largely attributed to focal iron deposition accompanying the plaques. It is believed that t...
Source: NeuroImage - Category: Neuroscience Source Type: research
PMID: 30758274 [PubMed - in process]
Source: Palliative Medicine - Category: Palliative Care Authors: Tags: Palliat Med Source Type: research
Niemann-Pick disease type C (NP-C) is a rare, progressive neurodegenerative disease caused by mutations in the NPC1 or the NPC2 gene. Neurocognitive deficits are common in NP-C, particularly in patients with the ...
Source: Orphanet Journal of Rare Diseases - Category: Internal Medicine Authors: Tags: Research Source Type: research
Both Abby, three, and Belle, seven, have Niemann-Pick Type C1, a genetic disease where in time, children slowly regress until they can ’t walk, eat, and dementia will set in before age 10.
Source: the Mail online | Health - Category: Consumer Health News Source Type: news
Hydroxypropyl-β-cyclodextrin formulated in nasal chitosan microspheres as candidate therapeutic agent in Alzheimer's disease. Curr Drug Deliv. 2017 Oct 19;: Authors: Rassu G, Gavini E, Carta A, Obinu A, Porcu EP, Giunchedi P Abstract Hydroxypropyl-β-cyclodextrin (HP-CD) is a hydroxyalkyl derivative of native β-cyclodextrin, cyclic oligosaccharide able to form inclusion complexes with many drugs and biomolecules [1]. Cyclodextrins have a possible neuroprotective effect due to their capability to extract and deplete cholesterol from cell membranes [1]. HP-CD is classified as an excipient ...
Source: Current Drug Delivery - Category: Drugs & Pharmacology Authors: Tags: Curr Drug Deliv Source Type: research
Conclusions: Diagnosis of ANCL remains challenging; expert pathologic analysis and recent molecular genetic advances revealed misdiagnoses in>1/3 of cases. We now have a refined group of cases that will facilitate identification of new causative genes.
Source: Neurology - Category: Neurology Authors: Tags: All Movement Disorders, All Cognitive Disorders/Dementia ARTICLE Source Type: research
Abstract Trisomy 21 and the consequent extra copy of the amyloid precursor protein (APP) gene and increased beta-amyloid (Aβ) peptide production underlie the universal development of Alzheimer's disease (AD) pathology and high risk of AD dementia in people with Down syndrome (DS). Trisomy 21 and other forms of aneuploidy also arise among neurons and peripheral cells in both sporadic and familial AD and in mouse and cell models thereof, reinforcing the conclusion that AD and DS are two sides of the same coin. The demonstration that 90% of the neurodegeneration in AD can be attributed to the selective loss of a...
Source: Current Alzheimer Research - Category: Neurology Authors: Tags: Curr Alzheimer Res Source Type: research
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