Anterior Temporal Atrophy and Posterior Progression in Patients with Parkinson's Disease.
Conclusions: Temporal pole atrophy as an early sign of PD is consistent with the PD pathology classification of Braak. The initial anterior temporal atrophy with spread to occipitotemporal and posterior parietal regions may subserve 'emotion-based' sensorimotor transformations and deficits in the visual domain, respectively, which may be regarded as premotor symptoms. © 2014 S. Karger AG, Basel. PMID: 25138823 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - August 16, 2014 Category: Neurology Authors: Potgieser AR, van der Hoorn A, Meppelink AM, Teune LK, Koerts J, de Jong BM Tags: Neurodegener Dis Source Type: research

Oligodendroglial Response in the Spinal Cord in TDP-43 Proteinopathy with Motor Neuron Involvement.
Conclusion: Our study further emphasizes the importance of oligodendroglia in the pathogenesis of TDP-43 proteinopathies with spinal cord involvement. © 2014 S. Karger AG, Basel. PMID: 25115814 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - August 5, 2014 Category: Neurology Authors: Rohan Z, Matej R, Rusina R, Kovacs GG Tags: Neurodegener Dis Source Type: research

Erratum.
Authors: PMID: 24970574 [PubMed - in process] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - July 3, 2014 Category: Neurology Tags: Neurodegener Dis Source Type: research

Pyroglutamate-Amyloid-β and Glutaminyl Cyclase Are Colocalized with Amyloid-β in Secretory Vesicles and Undergo Activity-Dependent, Regulated Secretion.
Conclusions: pGlu-Aβ and QC are present with Aβ in DCSV and undergo activity-dependent, regulated cosecretion with neurotransmitters. © 2014 S. Karger AG, Basel. PMID: 24943989 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - June 18, 2014 Category: Neurology Authors: Cynis H, Funkelstein L, Toneff T, Mosier C, Ziegler M, Koch B, Demuth HU, Hook V Tags: Neurodegener Dis Source Type: research

Protective Effects of Bexarotene against Amyloid-β25-35-Induced Dysfunction in Hippocampal Neurons through the Insulin Signaling Pathway.
Conclusion: The effects of bexarotene on Aβ-impaired excitability and sAP duration were mainly associated with K(+) channels through insulin signaling pathway, which may be an additional mechanism underlying the protective effect of bexarotene on AD. © 2014 S. Karger AG, Basel. PMID: 24802415 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - April 30, 2014 Category: Neurology Authors: Dai W, Yang J, Chen T, Yang Z Tags: Neurodegener Dis Source Type: research

Preface.
PMID: 24503527 [PubMed - in process] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - February 12, 2014 Category: Neurology Authors: Hanin I, Nitsch RM, Windisch M, Fisher A Tags: Neurodegener Dis Source Type: research

Author index vol. 13, no. 2-3, 2014.
Authors: PMID: 24503528 [PubMed - in process] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - February 12, 2014 Category: Neurology Tags: Neurodegener Dis Source Type: research

Electroencephalographic Upper/Low Alpha Frequency Power Ratio Relates to Cortex Thinning in Mild Cognitive Impairment.
ni GB Abstract Objective: Temporoparietal cortex thinning is associated with mild cognitive impairment (MCI) due to Alzheimer disease (AD). The increase in EEG upper/low α frequency power ratio has been associated with AD converter MCI subjects. We investigated the association of the EEG upper/low α frequency power ratio with patterns of cortical thickness in MCI. Methods: 74 adult subjects with MCI underwent clinical and neuropsychological evaluation, electroencephalography (EEG) recording and high-resolution 3-dimensional magnetic resonance imaging (MRI). The EEG upper/low α frequency power rat...
Source: Neuro-Degenerative Diseases - January 8, 2014 Category: Neurology Authors: Moretti DV, Paternicò D, Binetti G, Zanetti O, Frisoni GB Tags: Neurodegener Dis Source Type: research

Molecular Imaging in Sporadic Alzheimer's Disease Populations and Those Genetically at Risk.
Abstract The time course and order of the pathological-physiological processes in Alzheimer's disease (AD) are still under investigation and it is expected that molecular imaging will provide important insight into early brain pathology. Multi-tracer positron emission tomography studies visualizing fibrillar amyloid, inflammatory changes including astrocytosis and activation of microglia as well as cerebral glucose metabolism indicate that AD pathological processes are initiated and ongoing decades before the onset of cognitive symptoms. Therefore, prevention might be a new promising target for AD therapy. © ...
Source: Neuro-Degenerative Diseases - January 8, 2014 Category: Neurology Authors: Nordberg A Tags: Neurodegener Dis Source Type: research

We Can Treat Alzheimer's Disease Successfully in Mice but Not in Men: Failure in Translation? A Perspective.
Conclusion: The value of animal models depends mainly on the careful experimentation and correct interpretation of results. Appropriate planning of experiments will help to increase the predictive value in drug development programs, though this may also increase negative findings. However, the early failure may enable a faster focus on more promising strategies. © 2014 S. Karger AG, Basel. PMID: 24401335 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - January 7, 2014 Category: Neurology Authors: Windisch M Tags: Neurodegener Dis Source Type: research

White Matter Changes in Patients with Parkinson's Disease Carrying Small CGG Expansion FMR1 Alleles: A Pilot Study.
Conclusions: The results support our earlier claim that grey-zone alleles contribute to the severity of parkinsonism and white matter changes. © 2013 S. Karger AG, Basel. PMID: 24401315 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - December 24, 2013 Category: Neurology Authors: Trost N, Cook M, Hammersley E, Bui MQ, Brotchie P, Burgess T, Slater H, Storey E, Loesch DZ Tags: Neurodegener Dis Source Type: research

The Neuroprotection Exerted by Memantine, Minocycline and Lithium, against Neurotoxicity of CSF from Patients with Amyotrophic Lateral Sclerosis, Is Antagonized by Riluzole.
ero A, Martínez-Vila E, García AG Abstract In a recent study we found that cerebrospinal fluids (CSFs) from amyotrophic lateral sclerosis (ALS) patients caused 20-30% loss of cell viability in primary cultures of rat embryo motor cortex neurons. We also found that the antioxidant resveratrol protected against such damaging effects and that, surprisingly, riluzole antagonized its protecting effects. Here we have extended this study to the interactions of riluzole with 3 other recognized neuroprotective agents, namely memantine, minocycline and lithium. We found: (1) by itself riluzole exerted neurotox...
Source: Neuro-Degenerative Diseases - December 20, 2013 Category: Neurology Authors: Yáñez M, Matías-Guiu J, Arranz-Tagarro JA, Galán L, Viña D, Gómez-Pinedo U, Vela A, Guerrero A, Martínez-Vila E, García AG Tags: Neurodegener Dis Source Type: research

Prospective Flutemetamol Positron Emission Tomography and Histopathology in Normal Pressure Hydrocephalus.
Conclusions: [(18)F]Flutemetamol detects brain amyloid-β in vivo and shows promise as a valuable tool to study and possibly facilitate diagnosis of Alzheimer's disease both in patients with suspected NPH and among the wider population. © 2013 S. Karger AG, Basel. PMID: 24296542 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - November 27, 2013 Category: Neurology Authors: Rinne JO, Frantzen J, Leinonen V, Lonnrot K, Laakso A, Virtanen KA, Solin O, Kotkansalo A, Koivisto A, Sajanti J, Karppinen A, Lehto H, Rummukainen J, Buckley C, Smith A, Jones PA, Sherwin P, Farrar G, McLain R, Kailajarvi M, Grachev ID Tags: Neurodegener Dis Source Type: research

Environmental and Occupational Risk Factors for Amyotrophic Lateral Sclerosis: A Case-Control Study.
Conclusion: Workers exposed to metals and pesticides may be at greater risk of ALS. Future research should involve more accurate exposure assessment through the use of job exposure matrices, confirmation of occupation and biomarkers. © 2013 S. Karger AG, Basel. PMID: 24246552 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - November 12, 2013 Category: Neurology Authors: Malek AM, Barchowsky A, Bowser R, Heiman-Patterson T, Lacomis D, Rana S, Youk A, Stickler D, Lackland DT, Talbott EO Tags: Neurodegener Dis Source Type: research

The E280A Presenilin Mutation Reduces Voltage-Gated Sodium Channel Levels in Neuronal Cells.
Conclusion: Our data indicate that the FAD-linked PS1(E280A) mutation decreases Nav channel levels by partially inhibiting the PS/γ-secretase-mediated cleavage of Navβ2 in neuronal cells. © 2013 S. Karger AG, Basel. PMID: 24217025 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - November 5, 2013 Category: Neurology Authors: Kim DY, Wertz MH, Gautam V, D'Avanzo C, Bhattacharyya R, Kovacs DM Tags: Neurodegener Dis Source Type: research

Regulation of Autophagy by LRRK2 in Caenorhabditis elegans.
Conclusion: These results support a putative role of LRRK2 in the autophagic and mitochondrial systems. © 2013 S. Karger AG, Basel. PMID: 24192129 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - October 31, 2013 Category: Neurology Authors: Saha S, Liu-Yesucevitz L, Wolozin B Tags: Neurodegener Dis Source Type: research

Employing Alzheimer Disease Animal Models for Translational Research: Focus on Dietary Components.
Conclusions: Our findings support the beneficial effects of EVOO and highlight the possibility that continuous intake of high doses of OLE, both as a nutraceutical or as a food integrator, may prevent/delay the appearance of AD and reduce the severity of its symptoms. © 2013 S. Karger AG, Basel. PMID: 24192327 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - October 30, 2013 Category: Neurology Authors: Grossi C, Ed Dami T, Rigacci S, Stefani M, Luccarini I, Casamenti F Tags: Neurodegener Dis Source Type: research

Impact of Vitamin D on Amyloid Precursor Protein Processing and Amyloid-β Peptide Degradation in Alzheimer's Disease.
Impact of Vitamin D on Amyloid Precursor Protein Processing and Amyloid-β Peptide Degradation in Alzheimer's Disease. Neurodegener Dis. 2013 Oct 30; Authors: Grimm MO, Lehmann J, Mett J, Zimmer VC, Grösgen S, Stahlmann CP, Hundsdörfer B, Haupenthal VJ, Rothhaar TL, Herr C, Bals R, Grimm HS, Hartmann T Abstract Ninety percent of the elderly population has a vitamin D hypovitaminosis, and several lines of evidence suggest that there might be a potential causal link between Alzheimer's disease (AD) and a non-sufficient supply with vitamin D. However, the mechanisms linking AD to vitamin D have ...
Source: Neuro-Degenerative Diseases - October 30, 2013 Category: Neurology Authors: Grimm MO, Lehmann J, Mett J, Zimmer VC, Grösgen S, Stahlmann CP, Hundsdörfer B, Haupenthal VJ, Rothhaar TL, Herr C, Bals R, Grimm HS, Hartmann T Tags: Neurodegener Dis Source Type: research

Defining the Native State of α-Synuclein.
Defining the Native State of α-Synuclein. Neurodegener Dis. 2013 Oct 30; Authors: Selkoe D, Dettmer U, Luth E, Kim N, Newman A, Bartels T Abstract Misfolding and pathogenic aggregation of α-synuclein (αSyn) is a hallmark of familial and sporadic Parkinson's disease, but the physiological state of the protein in cells remains unsettled. We have further examined our hypothesis that endogenous αSyn can occur in normal cells as a metastable, helically folded tetramer, not solely as the unfolded monomer long thought to be its native form. At this meeting, we reviewed our recent approache...
Source: Neuro-Degenerative Diseases - October 30, 2013 Category: Neurology Authors: Selkoe D, Dettmer U, Luth E, Kim N, Newman A, Bartels T Tags: Neurodegener Dis Source Type: research

Effect of Donepezil in Alzheimer Disease Can Be Measured by a Computerized Human Analog of the Morris Water Maze.
Conclusions: The computerized hMWM has the potential to measure the effects of donepezil in mild AD. It is a sensitive cognitive outcome measure in AD clinical trials. © 2013 S. Karger AG, Basel. PMID: 24192578 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - October 30, 2013 Category: Neurology Authors: Hort J, Andel R, Mokrisova I, Gazova I, Amlerova J, Valis M, Coulson EJ, Harrison J, Windisch M, Laczó J Tags: Neurodegener Dis Source Type: research

Relative Ratio and Level of Amyloid-β 42 Surrogate in Cerebrospinal Fluid of Familial Alzheimer Disease Patients with Presenilin 1 Mutations.
Conclusion: A higher relative ratio of the CSF Aβ42 surrogate in PS1-FAD patients is not due to its increase in CSF, suggesting that massive Aβ42 accumulation in the PS1-FAD brain occurs without an apparent increase in Aβ42 secretion. © 2013 S. Karger AG, Basel. PMID: 24192669 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - October 30, 2013 Category: Neurology Authors: Tagami S, Okochi M, Yanagida K, Kodama T, Arai T, Kuwano R, Ikeuchi T, Takeda M Tags: Neurodegener Dis Source Type: research

Gamma-Secretase Inhibitor Activity of a Pterocarpus erinaceus Extract.
Conclusion: P. erinaceus extract appears as a new potent γ-secretase inhibitor selective towards APP processing. © 2013 S. Karger AG, Basel. PMID: 24192706 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - October 30, 2013 Category: Neurology Authors: Hage S, Marinangeli C, Stanga S, Octave JN, Quetin-Leclercq J, Kienlen-Campard P Tags: Neurodegener Dis Source Type: research

Sustained Alzheimer's Amyloid Pathology in Myeloid Differentiation Protein-88-Deficient APPswe/PS1 Mice.
Conclusion: Our findings indicate that impaired innate immune responses may play a role in AD pathology. © 2013 S. Karger AG, Basel. PMID: 24192711 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - October 30, 2013 Category: Neurology Authors: Goll Y, Bekenstein U, Barbash S, Greenberg DS, Zangen R, Shoham S, Soreq H Tags: Neurodegener Dis Source Type: research

The Therapeutic Effects of Human Adipose-Derived Stem Cells in Alzheimer's Disease Mouse Models.
Abstract Alzheimer's disease (AD) is an irreversible neurodegenerative disease, still lacking proper clinical treatment. Therefore, many researchers have focused on the possibility of therapeutic use of stem cells for AD. Adipose-derived stem cells (ASCs), mesenchymal stem cells (MSCs) isolated from adipose tissue, are well known for their pluripotency and their ability to differentiate into multiple tissue types and have immune modulatory properties similar to those of MSCs from other origins. Because of their biological properties, ASCs can be considered for cell therapy and neuroregeneration. Our recent results...
Source: Neuro-Degenerative Diseases - October 23, 2013 Category: Neurology Authors: Chang KA, Kim HJ, Joo Y, Ha S, Suh YH Tags: Neurodegener Dis Source Type: research

Modeling Amyotrophic Lateral Sclerosis in hSOD1 Transgenic Swine.
Abstract Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that occurs in two clinically indistinguishable forms: sporadic (SALS) and familial (FALS), the latter linked to several gene mutations, mostly inheritable in a dominant manner. Nearly 20% of FALS forms are linked to mutations in the Cu/Zn superoxide dismutase (SOD1) gene. Research on ALS relies on transgenic models and particularly on mice carrying a glycine-to-alanine conversion at the 93rd codon (G93A) of the hSOD1 gene. Although G93A transgenic mice have been widely employed in clinical trials and basic research, doubts have been...
Source: Neuro-Degenerative Diseases - October 23, 2013 Category: Neurology Authors: Chieppa MN, Perota A, Corona C, Grindatto A, Lagutina I, Vallino Costassa E, Lazzari G, Colleoni S, Duchi R, Lucchini F, Caramelli M, Bendotti C, Galli C, Casalone C Tags: Neurodegener Dis Source Type: research

Pyroglutamate-Modified Amyloid-β Protein Demonstrates Similar Properties in an Alzheimer's Disease Familial Mutant Knock-In Mouse and Alzheimer's Disease Brain.
Conclusion: This report supports a significant role for pE3Aβ in AD pathogenesis by confirming that pE3Aβ represents a large fraction of Aβ within the AD brain. Compared to the age-matched control brain, pE3Aβ increased to a greater extent compared to Aβ species without this N-terminal modification. Further, the APP/PS1-dKI model more closely resembles the AD brain in this regard, compared to the Tg2576 model. © 2013 S. Karger AG, Basel. PMID: 24158021 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - October 23, 2013 Category: Neurology Authors: Wu G, Miller RA, Connolly B, Marcus J, Renger J, Savage MJ Tags: Neurodegener Dis Source Type: research

Trials of Antidiabetic Drugs in Amyotrophic Lateral Sclerosis: Proceed with Caution?
Abstract Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder with limited therapeutic options. Clinical trials of several drugs shown to be effective in the superoxide dismutase (SOD1) model of ALS have shown no or negative effects when tested in humans. Here we discuss the role of pioglitazone, a peroxisome proliferator-activated receptor-γ agonist, which failed to show efficacy in a recently published phase II clinical trial of ALS patients. The antioxidant and anti-inflammatory properties of pioglitazone make it an attractive therapeutic candidate for neurodegenerative disorders. How...
Source: Neuro-Degenerative Diseases - October 2, 2013 Category: Neurology Authors: Jawaid A, Paganoni S, Hauser C, Schulz PE Tags: Neurodegener Dis Source Type: research

Function and Dysfunction of Presenilin.
Abstract The presenilin(PS) genes harbor approximately 90% of the identified mutations linked to familial forms of Alzheimer's disease, and the presenilin (PS) proteins are essential components of the γ-secretase complex involved in the proteolytic cleavage of type I receptors, such as Notch and the amyloid precursor protein. Genetic analysis employing cell type-specific conditional knockout technology highlighted the importance of PS in the adult brain, including learning and memory, synaptic function and age-dependent neuronal survival. In the central synapse, PS regulates neurotransmitter release, short- ...
Source: Neuro-Degenerative Diseases - October 2, 2013 Category: Neurology Authors: Shen J Tags: Neurodegener Dis Source Type: research

Imaging and Cerebrospinal Fluid Biomarkers in the Search for Alzheimer's Disease Mechanisms.
Conclusion: Our results indicate a heterogeneous biomarker expression, suggesting diversity of AD pathways in at-risk presymptomatic subjects. © 2013 S. Karger AG, Basel. PMID: 24107601 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - October 2, 2013 Category: Neurology Authors: Osorio RS, Pirraglia E, Gumb T, Mantua J, Ayappa I, Williams S, Mosconi L, Glodzik L, de Leon MJ Tags: Neurodegener Dis Source Type: research

Synthesis of Methoxy-X04 Derivatives and Their Evaluation in Alzheimer's Disease Pathology.
Conclusion: This resulted in the identification of new derivatives of methoxy-X04 with improved binding affinity. © 2013 S. Karger AG, Basel. PMID: 24080522 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - September 25, 2013 Category: Neurology Authors: Boländer A, Kieser D, Scholz C, Heyny-von Haußen R, Mall G, Goetschy V, Czech C, Schmidt B Tags: Neurodegener Dis Source Type: research

Familial Progressive Supranuclear Palsy: A Literature Review.
Conclusion: There is an increasing number of reported familial PSP. A recently performed genome-wide association study indicated genetic factors for this condition. Furthermore, clinical, pathological and genetic investigations will open new avenues to the discovery of causative genes and new therapeutics for PSP. © 2013 S. Karger AG, Basel. PMID: 24080486 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - September 24, 2013 Category: Neurology Authors: Fujioka S, Van Gerpen JA, Uitti RJ, Dickson DW, Wszolek ZK Tags: Neurodegener Dis Source Type: research

α-Synuclein in Parkinson's Disease: Pathogenic Function and Translation into Animal Models.
α-Synuclein in Parkinson's Disease: Pathogenic Function and Translation into Animal Models. Neurodegener Dis. 2013 Sep 24; Authors: Eschbach J, Danzer KM Abstract Parkinson's disease is a common neurodegenerative disease characterised by the loss of dopaminergic neurons in the substantia nigra pars compacta and the formation of α-synuclein aggregates found in Lewy bodies throughout the brain. Several α-synuclein transgenic mouse models have been generated, as well as viral-mediated overexpression of wild-type and mutated α-synuclein to mimic the disease and to delineate the pathogen...
Source: Neuro-Degenerative Diseases - September 24, 2013 Category: Neurology Authors: Eschbach J, Danzer KM Tags: Neurodegener Dis Source Type: research

The Inside-Out Amyloid Hypothesis and Synapse Pathology in Alzheimer's Disease.
eau M Abstract Cumulative evidence in brains and cultured neurons of Alzheimer's disease (AD) transgenic mouse models, as well as in human postmortem AD brains, highlights that age-related increases in β-amyloid peptide (Aβ), particularly in endosomes near synapses, are involved in early synapse dysfunction. Our immunoelectron microscopy and high-resolution immunofluorescence microscopy studies show that this early subcellular Aβ accumulation leads to progressive Aβ aggregation and pathology, particularly within dystrophic neurites and synapses. These studies confirm that neuritic/synaptic A&be...
Source: Neuro-Degenerative Diseases - September 24, 2013 Category: Neurology Authors: Gouras GK, Willén K, Faideau M Tags: Neurodegener Dis Source Type: research

Hippocampus-Related Cognitive Impairments in Young apoE4 Targeted Replacement Mice.
In conclusion, the present study shows that young apoE4 targeted replacement mice are impaired in numerous hippocampus-related learning and memory tasks. © 2013 S. Karger AG, Basel. PMID: 24080852 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - September 24, 2013 Category: Neurology Authors: Salomon-Zimri S, Boehm-Cagan A, Liraz O, Michaelson DM Tags: Neurodegener Dis Source Type: research

Can the Calcium Hypothesis Explain Synaptic Loss in Alzheimer's Disease?
Abstract Alzheimer's disease (AD) is the threat of modern humankind that is provoked by increased human lifespan. Despite extensive studies on AD pathology for more than 100 years, there are no disease-preventing therapies. Growing evidence suggests the role of calcium (Ca(2+)) in the pathogenesis of AD. The main purpose of the article is to understand whether modern science is able to explain the synapse loss observed in early AD and discuss the role of Ca(2+) hypothesis in it. Based on results obtained in our laboratory and others, we propose that familial AD-associated mutations in presenilins cause a Ca(2+) ov...
Source: Neuro-Degenerative Diseases - September 24, 2013 Category: Neurology Authors: Popugaeva E, Bezprozvanny I Tags: Neurodegener Dis Source Type: research

Allelic Interference: A Mechanism for Trans -Dominant Transmission of Loss of Function in the Neurodegeneration of Familial Alzheimer's Disease.
Abstract Presenilins (PSs) are catalytic components of the γ-secretase complexes that promote the ε-cleavage of cell surface proteins producing cytosolic peptides shown to function in cell signaling and gene expression. In addition, secretase cleavages at γ-sites of amyloid precursor protein substrates produce the amyloid-β (Aβ) peptides found in all people. Aggregation of Aβ peptides form the amyloid fibrils found in amyloid plaques of Alzheimer's disease (AD) patients and aged individuals. A common hypothesis suggests that AD is caused by aggregated Aβ peptides, but treatm...
Source: Neuro-Degenerative Diseases - September 24, 2013 Category: Neurology Authors: Robakis NK, Georgakopoulos A Tags: Neurodegener Dis Source Type: research

The Significance of α-Synuclein, Amyloid-β and Tau Pathologies in Parkinson's Disease Progression and Related Dementia.
Conclusion: The shared relevance of these pathologies in PD progression and dementia is in line with experimental data suggesting synergism between α-synuclein, tau and Aβ and with studies testing these proteins as disease biomarkers, hence favouring the eventual testing of therapeutic strategies targeting these proteins in PD. © 2013 S. Karger AG, Basel. PMID: 24028925 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - September 11, 2013 Category: Neurology Authors: Compta Y, Parkkinen L, Kempster P, Selikhova M, Lashley T, Holton JL, Lees AJ, Revesz T Tags: Neurodegener Dis Source Type: research

Cerebrospinal Fluid Tau Levels Predict Prognosis in Non-Inherited Frontotemporal Dementia.
Conclusions: This study argues that CSF tau levels may be considered in FTD to predict patients' outcome. Establishing in vivo prognostic biomarkers is mandatory to define homogeneous groups for inclusion in future clinical trials and to monitor the effectiveness of future therapeutic approaches. PMID: 24029600 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - September 11, 2013 Category: Neurology Authors: Borroni B, Benussi A, Cosseddu M, Archetti S, Padovani A Tags: Neurodegener Dis Source Type: research

Prefibrillar Tau Oligomers in Mild Cognitive Impairment and Alzheimer's Disease.
Conclusions: Clinicopathological findings highlight the importance of studying tau modifications in neuronal soma and neuritic processes, which may be the earliest pathological lesions that correlate with cognitive status. © 2013 S. Karger AG, Basel. PMID: 24029627 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - September 11, 2013 Category: Neurology Authors: Mufson EJ, Ward S, Binder L Tags: Neurodegener Dis Source Type: research

Parkin Plays a Role in Sporadic Parkinson's Disease.
Conclusion: Strategies aimed at maintaining parkin in a catalytically active state or interfering with the toxicity of AIMP2 and PARIS (ZNF746) offer new therapeutic opportunities. © 2013 S. Karger AG, Basel. PMID: 24029689 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - September 11, 2013 Category: Neurology Authors: Dawson TM, Dawson VL Tags: Neurodegener Dis Source Type: research

Tau Immunotherapy and Imaging.
Abstract Disappointing findings from recent phase III trials on amyloid-β (Aβ) immunotherapy for Alzheimer's disease (AD) have shifted the focus of such treatments to the tau protein. As tau pathology correlates better with the degree of dementia than Aβ plaque burden, it is a more attractive target once cognitive impairments are evident, while Aβ therapies may be better suited for the presymptomatic phase of the disease. Over 12 years ago, we initiated a tau immunotherapy program, seeking to alleviate the functional impairments associated with tau lesions in tauopathies. We have reported that ...
Source: Neuro-Degenerative Diseases - September 11, 2013 Category: Neurology Authors: Sigurdsson EM Tags: Neurodegener Dis Source Type: research

Neuroinflammatory Phenotypes and Their Roles in Alzheimer's Disease.
Conclusion: Heterogeneity in the neuroinflammatory state of the early AD brain may represent a source of variability among the AD population. It may also reflect the presence of comorbidities. © 2013 S. Karger AG, Basel. PMID: 24021538 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - September 6, 2013 Category: Neurology Authors: Wilcock DM Tags: Neurodegener Dis Source Type: research

MK886 Reduces Cerebral Amyloid Angiopathy Severity in TgCRND8 Mice.
Conclusion: These data suggest that 5-LOX and FLAP inhibitors may be useful in the treatment of CAA and AD. PMID: 24021653 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - September 6, 2013 Category: Neurology Authors: Hawkes CA, Shaw JE, Brown M, Sampson AP, McLaurin J, Carare RO Tags: Neurodegener Dis Source Type: research

SOD1-G93A Mice Exhibit Muscle-Fiber-Type-Specific Decreases in Glucose Uptake in the Absence of Whole-Body Changes in Metabolism.
Conclusions: These results suggest that alterations in muscle metabolism occur in a fiber-type-specific manner in ALS, but do not necessarily lead to whole-body metabolic changes in SOD1-G93A mice. © 2013 S. Karger AG, Basel. PMID: 24021858 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - September 6, 2013 Category: Neurology Authors: Smittkamp SE, Morris JK, Bomhoff GL, Chertoff ME, Geiger PC, Stanford JA Tags: Neurodegener Dis Source Type: research

Immune Activation in Amyloid-β-Related Angiitis Correlates with Decreased Parenchymal Amyloid-β Plaque Load.
Conclusion: Our results are in line with the hypothesis of ABRA being the result of an excessive immune response to Aβ and show that this can lead to enhanced clearance of Aβ from the brain parenchyma by immune-mediated mechanisms. © 2013 S. Karger AG, Basel. PMID: 24021982 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - September 6, 2013 Category: Neurology Authors: Bogner S, Bernreuther C, Matschke J, Barrera-Ocampo A, Sepulveda-Falla D, Leypoldt F, Magnus T, Haag F, Bergmann M, Brück W, Vogelgesang S, Glatzel M Tags: Neurodegener Dis Source Type: research

Progranulin Peripheral Levels as a Screening Tool for the Identification of Subjects with Progranulin Mutations in a Portuguese Cohort.
Conclusion: This study supports the use of a novel assay for the determination of PGRN levels as a screening procedure to identify patients harboring null PGRN mutations. This approach would significantly decrease the required PGRN mutation analysis workload and should be extended to other clinical phenotypes than behavioral variant frontotemporal dementia and to apparently sporadic cases. PMID: 24022032 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - September 6, 2013 Category: Neurology Authors: Almeida MR, Baldeiras I, Ribeiro MH, Santiago B, Machado C, Massano J, Guimarães J, Resende Oliveira C, Santana I Tags: Neurodegener Dis Source Type: research

Effect of Proinflammatory Gene Polymorphisms on the Risk of Alzheimer's Disease.
Conclusion: Proinflammatory genotypes might influence the development and progression of AD exerting a potential synergistic effect. PMID: 24022074 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - September 6, 2013 Category: Neurology Authors: Flex A, Giovannini S, Biscetti F, Liperoti R, Spalletta G, Straface G, Landi F, Angelini F, Caltagirone C, Ghirlanda G, Bernabei R Tags: Neurodegener Dis Source Type: research

Influence of Single Nucleotide Polymorphisms in COMT , MAO-A and BDNF Genes on Dyskinesias and Levodopa Use in Parkinson's Disease.
We examined the influence of SNPs in the catechol-O-methyltransferase (COMT), monoamine oxidase A (MAO-A) and brain-derived neurotrophic factor (BDNF) genes on LID in a cohort of 285 pathologically confirmed Parkinson's disease patients, using data from their complete disease course. Results: Dyskinetic patients demonstrated younger age at disease onset (60.3 vs. 66.4 years, p
Source: Neuro-Degenerative Diseases - September 5, 2013 Category: Neurology Authors: Cheshire P, Bertram K, Ling H, O'Sullivan SS, Halliday G, McLean C, Bras J, Foltynie T, Storey E, Williams DR Tags: Neurodegener Dis Source Type: research

Interplay between Parkin and p53 Governs a Physiological Homeostasis That Is Disrupted in Parkinson's Disease and Cerebral Cancer.
Abstract Parkin is responsible for most autosomal juvenile recessive cases of Parkinson's disease (PD). Besides its well-characterized function as ubiquitin ligase, we previously established that parkin could repress p53 at the transcriptional level. Interestingly, p53 was recently shown to upregulate parkin, suggesting a feedback loop by which parkin and p53 interplay, thereby contributing to their physiological homeostasis. This equilibrium is disrupted in both PD and cerebral cancer. Thus, when parkin is mutated in PD, its transcriptional ability to repress p53 is abolished. Therefore, p53 elevation could likel...
Source: Neuro-Degenerative Diseases - September 4, 2013 Category: Neurology Authors: Checler F, Alves da Costa C Tags: Neurodegener Dis Source Type: research

Cytoskeletal Protection: Acting on Notch to Prevent Neuronal Dysfunction.
Abstract Functional and structural plasticity is a fundamental property of the brain involving chemical, electrical, molecular and cellular responses and leading to reorganization of connections within a brain region and/or between brain regions. The Notch pathway has been recognized as one of the main contributors in regulating neural development and has been proposed as a key mediator in neuroplasticity. We supported this concept, demonstrating that Notch plays a role in determining the only possible 'cell fate' decisions in post-mitotic mature neurons: synaptic remodelling or neurite extension/retraction. We de...
Source: Neuro-Degenerative Diseases - September 4, 2013 Category: Neurology Authors: Bonini SA, Ferrari-Toninelli G, Maccarinelli G, Bettinsoli P, Montinaro M, Memo M Tags: Neurodegener Dis Source Type: research