Loss of laforin or malin results in increased Drp1 level and concomitant mitochondrial fragmentation in Lafora disease mouse models.

Loss of laforin or malin results in increased Drp1 level and concomitant mitochondrial fragmentation in Lafora disease mouse models. Neurobiol Dis. 2017 Jan 04;: Authors: Upadhyay M, Agarwal S, Bhadauriya P, Ganesh S Abstract Lafora disease (LD) is an autosomal recessive form of a fatal disorder characterized by the myoclonus epilepsy, ataxia, psychosis, dementia, and dysarthria. A hallmark of LD is the presence of abnormal glycogen inclusions called Lafora bodies in the affected tissues including the neurons. LD can be caused by defects either in the laforin phosphatase coded by the EPM2A gene or in the malin E3 ubiquitin ligase coded by the NHLRC1 gene. The mouse models of LD, created by the targeted disruption of the LD genes, display several neurodegenerative changes. Prominent among them are the autophagic defects, abnormally large lysosomes, neurofibrillary tangles, amyloid beta deposits, and abnormal mitochondria. However, whether or not such neurodegenerative changes are a direct effect of the loss of laforin/malin was not unequivocally established. Here, we show that laforin- or malin-deficient neurons and fibroblasts display a significantly higher number of fragmented mitochondria. Loss of laforin or malin resulted in increased levels of the mitochondrial fission GTPase Drp1, its enhanced mitochondrial targeting, and increased intracellular calcium levels. Intriguingly, laforin or malin display opposite effects on the cellular level parkin, an ...
Source: Neurobiology of Disease - Category: Neurology Authors: Tags: Neurobiol Dis Source Type: research

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In conclusion, the DEGs and hub genes identified in the present study may be used to understand the molecular pathways involved in the initiation and progression of malignant melanoma. Furthermore, the present study may aid in the identification of possible targets for the diagnosis and treatment of melanoma. PMID: 31620197 [PubMed]
Source: Oncology Letters - Category: Cancer & Oncology Tags: Oncol Lett Source Type: research
This article is part of a Special Issue entitled: Cardiomyocyte biology: new pathways of differentiation and regeneration edited by Marijke Brink, Marcus C. Schaub, and Christian Zuppinger.
Source: Biochimica et Biophysica Acta (BBA) Molecular Cell Research - Category: Molecular Biology Source Type: research
Publication date: Available online 17 October 2019Source: Carbohydrate PolymersAuthor(s): Ahmed Salama, Ragab E. Abou-Zeid, Iriczalli Cruz-Maya, Vincenzo GuarinoAbstractTEMPO oxidized cellulose nanofibers (T-CNF) were prepared from cellulose pulp which is extracted from bagasse. Soy protein hydrolysate (SPH) was grafted on T-CNF via amidation of carboxylic groups. Biomineralization was, then, assessed via calcium phosphates (CaP) precipitation in twice-simulated body fluid until formation of a new bioactive material. Protein was efficiently grafted without alteration of morphology and nanofibrils packing as reported by Fou...
Source: Carbohydrate Polymers - Category: Biomedical Science Source Type: research
This study examined whether a history of traumatic brain injury (TBI) is associated with age at onset of Alzheimer's disease (AD) in three racial-ethnic groups. METHODS: Data from 7,577 non-Hispanic Caucasian, 792 African American, and 870 Hispanic participants with clinically diagnosed AD were obtained from the National Alzheimer's Coordinating Center. Participants were categorized by the presence or absence of self-reported remote history of TBI (>1 year before diagnosis of AD) with loss of consciousness (LOC) (TBI+) or no history of TBI with LOC (TBI-). Any group differences in education; sex; APOE ε4 all...
Source: Journal of Neuropsychiatry and Clinical Neurosciences - Category: Psychiatry Tags: J Neuropsychiatry Clin Neurosci Source Type: research
Publication date: Available online 17 October 2019Source: Chemico-Biological InteractionsAuthor(s): Lili Zhang, Xiaogui Qi, Guowei Zhang, Yingying Zhang, Jiali TianAbstractType II diabetes is recognized as a major risk factor for death due to cardiovascular complications such as coronary heart disease (CHD), but the complex interplay between these two diseases remains poorly understood. Suppression of oxidative stress, apoptosis, and inflammation of endothelial cells is a valuable treatment strategy to prevent or halt the progression of CHD. In the present study, we used real-time polymerase chain reaction (PCR), Western b...
Source: Chemico Biological Interactions - Category: Biochemistry Source Type: research
We present the results of force field molecular dynamics simulations of calcium aluminosilicate, sodium aluminosilicate, calcium silicate, and sodium silicate liquids under temperature gradient conditions. Non-equilibrium molecular dynamics simulations of these liquids under temperature gradient conditions reproduced the trend reported for Soret coefficients, with network-forming cations having larger coefficients compared with network-modifying ones. We found a linear relationship between the Soret coefficients and the variance of the potential energy distribution of each cation, which corresponds to the activation energy...
Source: Journal of Non Crystalline Solids - Category: Chemistry Source Type: research
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Source: The Journal of Alzheimers Association - Category: Psychiatry Tags: Alzheimers Dement Source Type: research
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Source: Molecular Genetics and Metabolism - Category: Genetics & Stem Cells Source Type: research
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Source: NeuroImage: Clinical - Category: Radiology Source Type: research
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Source: NeuroImage: Clinical - Category: Radiology Source Type: research
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