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

Stress Impairs Synaptic Plasticity in Triple-Transgenic Alzheimer's Disease Mice: Rescue by Ryanodine.
Conclusions: Stress experiences in young adults may accelerate the cognitive loss in AD mice, adding another dimension to the plethora of factors that lead to AD. © 2013 S. Karger AG, Basel. PMID: 24008840 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - September 4, 2013 Category: Neurology Authors: Grigoryan G, Biella G, Albani D, Forloni G, Segal M Tags: Neurodegener Dis Source Type: research

ADAM10 in Synaptic Physiology and Pathology.
Conclusions: Regulated interaction of ADAM10 with SAP97 and AP2 discloses a novel physiological mechanism of ADAM10 activity regulation at the synapses. This phenomenon produces a situation whereby synaptically regulated ADAM10 activity is positioned to modulate synaptic functioning. © 2013 S. Karger AG, Basel. PMID: 24008925 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - September 4, 2013 Category: Neurology Authors: Musardo S, Marcello E, Gardoni F, Di Luca M Tags: Neurodegener Dis Source Type: research

Resting-State Networks in Alzheimer's Disease and Parkinson's Disease.
Abstract While there is good evidence for altered resting-state networks, particularly the default mode network (DMN), in both Alzheimer's disease (AD) and amnestic mild cognitive impairment preceding AD, there are rather conflicting data on changes in the DMN in Parkinson's disease (PD) and PD with cognitive impairment. This paper will focus on DMN study results, particularly in PD, as assessed by functional MRI. © 2013 S. Karger AG, Basel. PMID: 24008996 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - September 4, 2013 Category: Neurology Authors: Rektorova I Tags: Neurodegener Dis Source Type: research

Functional Interaction between Amyloid-β Precursor Protein and Peripherin Neurofilaments: A Shared Pathway Leading to Alzheimer's Disease and Amyotrophic Lateral Sclerosis?
Conclusion: Our results indicate that a fraction of APP is cleaved by β-secretase in the soma and that the generated sAPP becomes associated with perinuclear peripherin neurofilaments. These findings link the metabolism of APP - which is dysregulated in AD - to the organization of neurofilaments - which is abnormal in ALS - and suggest a possible crosstalk/overlap between the molecular mechanisms of these diseases. PMID: 24009040 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - September 4, 2013 Category: Neurology Authors: Muresan V, Villegas C, Ladescu Muresan Z Tags: Neurodegener Dis Source Type: research

Comparing Predictors of Conversion to Alzheimer's Disease Using the Disease State Index.
Conclusion: In addition to neuropsychological testing, MRI is recommended to be included for differentiating S-MCI from P-MCI. APOE genotype, CSF and PET may provide some additional information. © 2013 S. Karger AG, Basel. PMID: 23969422 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - August 22, 2013 Category: Neurology Authors: Muñoz-Ruiz MA, Hall A, Mattila J, Koikkalainen J, Herukka SK, Vanninen R, Liu Y, Lötjönen J, Soininen H Tags: Neurodegener Dis Source Type: research

Possible Role of Dynorphins in Alzheimer's Disease and Age-Related Cognitive Deficits.
Conclusions: These results suggest that elevated dynorphin expression occurring during aging and AD may mediate cognitive deficits by altering the glutamatergic system integrity. © 2013 S. Karger AG, Basel. PMID: 23970097 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - August 22, 2013 Category: Neurology Authors: Ménard C, Herzog H, Schwarzer C, Quirion R Tags: Neurodegener Dis Source Type: research

Course of Irritability, Depression and Apathy in Huntington's Disease in Relation to Motor Symptoms during a Two-Year Follow-Up Period.
Conclusion: An increase in motor symptoms in the at baseline premotor symptomatic mutation carriers is related to an increase in irritability, which may be an early and sensitive marker for disease progression. PMID: 23948661 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - August 16, 2013 Category: Neurology Authors: van Duijn E, Reedeker N, Giltay EJ, Eindhoven D, Roos RA, van der Mast RC Tags: Neurodegener Dis Source Type: research

Valproic Acid Attenuates Disease Symptoms and Increases Endogenous Myelin Repair by Recruiting Neural Stem Cells and Oligodendrocyte Progenitors in Experimental Autoimmune Encephalomyelitis.
Conclusions: Results suggest VPA as a potential therapy for remyelinating the lesions in MS and for faster recovery from disease relapses. The effect of VPA seems to be mediated by endogenous progenitors recruitment. PMID: 23949302 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - August 16, 2013 Category: Neurology Authors: Pazhoohan S, Satarian L, Asghari AA, Salimi M, Kiani S, Mani AR, Javan M Tags: Neurodegener Dis Source Type: research

β-Amyloid Precursor Protein: Function in Stem Cell Development and Alzheimer's Disease Brain.
β-Amyloid Precursor Protein: Function in Stem Cell Development and Alzheimer's Disease Brain. Neurodegener Dis. 2013 Aug 7; Authors: Small DH, Hu Y, Bolós M, Dawkins E, Foa L, Young KM Abstract Stem cell therapy may be a suitable approach for the treatment of many neurodegenerative diseases. However, one major impediment to the development of successful cell-based therapies is our limited understanding of the mechanisms that instruct neural stem cell behaviour, such as proliferation and cell fate specification. The β-amyloid precursor protein (APP) of Alzheimer's disease (AD) may play an i...
Source: Neuro-Degenerative Diseases - August 7, 2013 Category: Neurology Authors: Small DH, Hu Y, Bolós M, Dawkins E, Foa L, Young KM Tags: Neurodegener Dis Source Type: research

Imaging and Cognitive Reserve Studies Predict Dementia in Presymptomatic Alzheimer's Disease Subjects.
Abstract There is strong evidence that Alzheimer's disease (AD) pathology starts decades before clinical onset. Cognitive reserve (CR) and brain reserve can be a good predictive model for AD development. Neuroimaging can help in describing cerebral reserves, as well as in detecting AD brain pathology before the onset of clinical dementia. Education and occupation act as proxies for CR and are associated with a lower risk of AD and delayed onset of symptoms. The apolipoprotein E (ApoE)-ε4 allele is a strong risk factor for AD and is associated with lower hippocampal volume even in normal aging. A fluorodeox...
Source: Neuro-Degenerative Diseases - August 7, 2013 Category: Neurology Authors: Ferrari C, Nacmias B, Bagnoli S, Piaceri I, Lombardi G, Pradella S, Tedde A, Sorbi S Tags: Neurodegener Dis Source Type: research

How Can We Improve Transfer of Outcomes from Randomized Clinical Trials to Clinical Practice with Disease-Modifying Drugs in Alzheimer's Disease?
Conclusions: Global clinical scales such as CDR-SB and ROSA should be considered for use with treatments aiming at slowing disease progression. © 2013 S. Karger AG, Basel. PMID: 23942173 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - August 7, 2013 Category: Neurology Authors: Gauthier S, Leuzy A, Rosa-Neto P Tags: Neurodegener Dis Source Type: research

Inhibition of Amyloid Precursor Protein Processing Leads to Downregulation of Apoptotic Genes in Alzheimer's Disease Animal Models.
Conclusion: A therapy that is capable of reducing not only the direct hallmarks of AD but also the components that lead to neuronal apoptosis might have neuroprotective potential in AD treatment. © 2013 S. Karger AG, Basel. PMID: 23942195 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - August 7, 2013 Category: Neurology Authors: Rabinovich-Nikitin I, Solomon B Tags: Neurodegener Dis Source Type: research

MRI for Iron Mapping in Alzheimer's Disease.
Conclusions: MRI allows to quantitatively map iron accumulation in the brain. The clinical significance of increased iron levels in AD needs to be determined in both cross-sectional and longitudinal studies. The advent of ultra-high field imaging in clinical applications will increase image resolution and will allow in vivo iron detection in neocortical structures in AD patients both cross-sectionally and longitudinally. © 2013 S. Karger AG, Basel. PMID: 23942230 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - August 7, 2013 Category: Neurology Authors: Langkammer C, Ropele S, Pirpamer L, Fazekas F, Schmidt R Tags: Neurodegener Dis Source Type: research

Quantitative Profiling and Identification of Plasma Proteins of Spinocerebellar Ataxia Type 2 Patients.
Conclusions: Some of these identified proteins in SCA2 can be useful for therapeutic, diagnostic and prognostic purposes. PMID: 23735416 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - May 31, 2013 Category: Neurology Authors: Swarup V, Srivastava AK, Padma MV, Moganty RR Tags: Neurodegener Dis Source Type: research

Diffusion Tensor Imaging in Idiopathic Parkinson's Disease and Multisystem Atrophy (Parkinsonian Type).
Conclusion: Changes of the ADC close to the putamen proved most consistent and seem to be promising for the ongoing clinical implementation of DTI for the differentiation of hypokinetic-rigid movement disorders. PMID: 23711586 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - May 24, 2013 Category: Neurology Authors: Cnyrim CD, Kupsch A, Ebersbach G, Hoffmann KT Tags: Neurodegener Dis Source Type: research

Substantia Nigra Hyperechogenicity as a Marker for Parkinson's Disease: A Population-Based Study.
Conclusion: SN hyperechogenicity may represent a useful biomarker for PD not only in a hospital-based setting but also in the general community. PMID: 23689066 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - May 18, 2013 Category: Neurology Authors: Mahlknecht P, Seppi K, Stockner H, Nocker M, Scherfler C, Kiechl S, Willeit J, Schmidauer C, Gasperi A, Rungger G, Poewe W Tags: Neurodegener Dis Source Type: research

Molecular Characterization of X-Linked Adrenoleukodystrophy in a Tunisian Family: Identification of a Novel Missense Mutation in the ABCD1 Gene.
Conclusion: Using bioinformatic tools we suggest that this novel variant may have deleterious effects on adrenoleukodystrophy protein structure and function. PMID: 23651979 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - May 3, 2013 Category: Neurology Authors: Kallabi F, Hadj Salem I, Ben Salah G, Ben Turkia H, Ben Chehida A, Tebib N, Fakhfakh F, Kamoun H Tags: Neurodegener Dis Source Type: research

A Hypnic Hypothesis of Alzheimer's Disease.
Conclusion: The formation of a dynamic toxic state within this vulnerable network linked to sleep-wake disruption, would in turn lead to failure of synaptic repair, increased transmission of pathogenic misfolded proteins and a self-amplifying neurodegenerative process. We consider the evidence for this hypnic hypothesis and the implications that follow on from it. PMID: 23635607 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - April 26, 2013 Category: Neurology Authors: Clark CN, Warren JD Tags: Neurodegener Dis Source Type: research

Postmortem Brain Levels of Urate and Precursors in Parkinson's Disease and Related Disorders.
Conclusions: Though limited in sample size, these findings lend support to the inverse association between urate levels and PD, as well as possibly AD. The finding of increased urate in DLB brain tissue is novel and warrants further study. PMID: 23467193 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - February 28, 2013 Category: Neurology Authors: McFarland NR, Burdett T, Desjardins CA, Frosch MP, Schwarzschild MA Tags: Neurodegener Dis Source Type: research

DNA Damage in Alzheimer Disease Lymphocytes and Its Relation to Premature Centromere Division.
ć VP Abstract While Alzheimer disease (AD) is considered a neurodegenerative disorder, the importance of chromosome instability in non-neuronal cells is equally important, not only for shedding light on the etiology of the disease, but also for possible diagnostic purposes and monitoring the progress of the disease. Here, we evaluated the frequency of DNA damage and expression of premature centromere division (PCD) in peripheral blood lymphocytes of sporadic AD patients, age-matched and young controls. The results show that in male patients with AD, the frequencies of PCD and DNA damage were significantly greater (88%, p
Source: Neuro-Degenerative Diseases - February 13, 2013 Category: Neurology Authors: Zivković L, Spremo-Potparević B, Siedlak SL, Perry G, Plećaš-Solarović B, Milićević Z, Bajić VP Tags: Neurodegener Dis Source Type: research

Neuropathology of Partial PGC-1α Deficiency Recapitulates Features of Mitochondrial Encephalopathies but Not of Neurodegenerative Diseases.
Conclusion: We conclude that the lack of FL-PGC-1α per se is insufficient to recapitulate major features of neurodegenerative diseases, but evokes a pathology seen in mitochondrial encephalopathies, which makes PGC-1α-deficient mice a valuable model for this yet incurable group of diseases. PMID: 23406886 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - February 13, 2013 Category: Neurology Authors: Szalardy L, Zadori D, Plangar I, Vecsei L, Weydt P, Ludolph AC, Klivenyi P, Kovacs GG Tags: Neurodegener Dis Source Type: research

Phenotypes in Swiss Patients with Familial ALS Carrying TARDBP Mutations.
Conclusion: In this Swiss population, the frequency of familial ALS is higher than reported earlier in other populations. The novel p.Gly376Asp TARDBP mutation is associated with rapid disease progression and may be associated with an accessory nipple while the p.Asn352Ser mutation is associated with slow disease progression. PMID: 23327806 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - January 10, 2013 Category: Neurology Authors: Czell D, Andersen PM, Morita M, Neuwirth C, Perren F, Weber M Tags: Neurodegener Dis Source Type: research