Mitochondrial dysfunction in neurons in Friedreich's ataxia
Publication date: Available online 23 November 2019Source: Molecular and Cellular NeuroscienceAuthor(s): Anna Stepanova, Jordi MagranéAbstractFriedreich's ataxia is a multisystemic genetic disorder within the family of mitochondrial diseases that is characterized by reduced levels of the essential mitochondrial protein frataxin. Based on clinical evidence, the peripheral nervous system is affected early, neuronal dysfunction progresses towards the central nervous system, and other organs (such as heart and pancreas) are affected later. However, little attention has been given to the specific aspects of mitochondria functi...
Source: Molecular and Cellular Neuroscience - November 25, 2019 Category: Neuroscience Source Type: research

Editorial Board
Publication date: December 2019Source: Molecular and Cellular Neuroscience, Volume 101Author(s): (Source: Molecular and Cellular Neuroscience)
Source: Molecular and Cellular Neuroscience - November 24, 2019 Category: Neuroscience Source Type: research

Editorial Board
Publication date: October 2019Source: Molecular and Cellular Neuroscience, Volume 100Author(s): (Source: Molecular and Cellular Neuroscience)
Source: Molecular and Cellular Neuroscience - November 19, 2019 Category: Neuroscience Source Type: research

Overexpression of human wtTDP-43 causes impairment in hippocampal plasticity and behavioral deficits in CAMKII-tTa transgenic mouse model
ConclusionsOverall, our findings suggest the significant vulnerability of hippocampal neurons toward elevated wtTDP-43 levels possibly via PCP-4 and GluR-dependent calcium signaling pathways. Further, we report that wtTDP-43 expression induced selective CA2 subfield degeneration, contributing to the deterioration of the hippocampal-dependent cognitive phenotype. (Source: Molecular and Cellular Neuroscience)
Source: Molecular and Cellular Neuroscience - November 7, 2019 Category: Neuroscience Source Type: research

Glial mitochondrial function and dysfunction in health and neurodegeneration
Publication date: Available online 31 October 2019Source: Molecular and Cellular NeuroscienceAuthor(s): Kevin McAvoy, Hibiki KawamataAbstractMitochondria play essential metabolic roles in neural cells. Mitochondrial dysfunction has profound effects on the brain. In primary mitochondrial diseases, mutations that impair specific oxidative phosphorylation (OXPHOS) proteins or OXPHOS assembly factors lead to isolated biochemical defects and a heterogeneous group of clinical phenotypes, including mitochondrial encephalopathies. A broader defect of OXPHOS function, due to mutations in proteins involved in mitochondrial DNA maint...
Source: Molecular and Cellular Neuroscience - November 2, 2019 Category: Neuroscience Source Type: research

Hippocampal stimulation promotes intracellular Tip60 dynamics with concomitant genome reorganization and synaptic gene activation
Publication date: Available online 1 November 2019Source: Molecular and Cellular NeuroscienceAuthor(s): Ashley Karnay, Bhanu Chandra Karisetty, Mariah Beaver, Felice ElefantAbstractGenomic reorganizations mediating the engagement of target genes to transcription factories (TFs), characterized as specialized nuclear subcompartments enriched in hyperphosphorylated RNA polymerase II (RNAPII) and transcriptional regulators, act as an important layer of control in coordinating efficient gene transcription. However, their presence in hippocampal neurons and potential role in activity-dependent coregulation of genes within the br...
Source: Molecular and Cellular Neuroscience - November 2, 2019 Category: Neuroscience Source Type: research

Fibroblast growth Factor-21 promotes ketone body utilization in neurons through activation of AMP-dependent kinase
Publication date: Available online 29 October 2019Source: Molecular and Cellular NeuroscienceAuthor(s): Yurika Katsu-Jiménez, Alfredo Giménez-CassinaAbstractEnergy supply to the brain is essential to ensure correct neuronal function, and glucose is the main fuel utilized by neurons. In metabolically challenging situations when glucose availability is restricted, brain cells may switch to alternative carbon substrates. This ensures energy supply to preserve the functions of the central nervous system. In this regard, ketone bodies, a by-product of fat metabolism, play a key role. They can replace glucose as the main sourc...
Source: Molecular and Cellular Neuroscience - October 30, 2019 Category: Neuroscience Source Type: research

Role of GPCR signaling and calcium dysregulation in Alzheimer's disease
Publication date: Available online 23 October 2019Source: Molecular and Cellular NeuroscienceAuthor(s): Sushma, Amal Chandra MondalAbstractAlzheimer's disease (AD), a late onset neurodegenerative disorder is characterized by the loss of memory, disordered cognitive function, caused by accumulation of amyloid-β (Aβ) peptide and neurofibrillary tangles (NFTs) in the neocortex and hippocampal brain area. Extensive research has been done on the findings of the disease etiology or pathological causes of aggregation of Aβ and hyperphosphorylation of tau protein without much promising results. Recently, calcium dysregulation h...
Source: Molecular and Cellular Neuroscience - October 24, 2019 Category: Neuroscience Source Type: research

Ginkgolic acid promotes autophagy-dependent clearance of intracellular alpha-synuclein aggregates
In this study, the effect on α-syn aggregate clearance of ginkgolic acid (GA), a natural compound extracted from Ginkgo biloba leaves that inhibits SUMOylation amongst other pathways, was assessed in SH-SY5Y neuroblastoma cells and rat primary cortical neurons. Depolarization of SH-SY5Y neuroblastoma cells and rat primary cortical neurons with KCl was used to induce α-syn aggregate formation. Cells pre-treated with either GA or the related compound, anacardic acid, revealed a significant decrease in intracytoplasmic aggregates immunopositive for α-syn and SUMO-1. An increased frequency of autophagosomes was also detecte...
Source: Molecular and Cellular Neuroscience - October 24, 2019 Category: Neuroscience Source Type: research

CBP and p300 coactivators contribute to the maintenance of Isl1 expression by the Onecut transcription factors in embryonic spinal motor neurons
Publication date: Available online 21 October 2019Source: Molecular and Cellular NeuroscienceAuthor(s): Mathilde Toch, Frédéric ClotmanAbstractOnecut transcription factors are required to maintain Islet1 (Isl1) expression in developing spinal motor neurons (MNs), and this process is critical for proper MN differentiation. However, the mechanisms whereby OC stimulate Isl1 expression remain unknown. CREB-binding protein (CBP) and p300 paralogs are transcriptional coactivators that interact with OC proteins in hepatic cells. In the embryonic spinal cord, CBP and p300 play key roles in neurogenesis and MN differentiation. He...
Source: Molecular and Cellular Neuroscience - October 22, 2019 Category: Neuroscience Source Type: research

L-type Ca2+ channels and charybdotoxin-sensitive Ca2+-activated K+ channels are required for reduction of GABAergic activity induced by β2-adrenoceptor in the prefrontal cortex
Publication date: Available online 20 October 2019Source: Molecular and Cellular NeuroscienceAuthor(s): Wei-Ke Deng, Xing Wang, Hou-Cheng Zhou, Fei LuoAbstractWhereas β2-adrenoceptor (β2-AR) has been reported to reduce GABAergic activity in the prefrontal cortex (PFC), the underlying cellular and molecular mechanisms have not been completely determined. Here, we showed that β2-AR agonist Clenbuterol (Clen) decreased GABAergic transmission onto PFC layer V/VI pyramidal neurons via a presynaptic mechanism without altering postsynaptic GABA receptors. Clen decreased the action potential firing rate but increased the burst ...
Source: Molecular and Cellular Neuroscience - October 21, 2019 Category: Neuroscience Source Type: research

Crosstalk between Nrf2 signaling and mitochondrial function in Parkinson's disease
Publication date: Available online 20 October 2019Source: Molecular and Cellular NeuroscienceAuthor(s): Navneet Ammal Kaidery, Manuj Ahuja, Bobby ThomasAbstractSearch for a definitive cure for neurodegenerative disorders like Parkinson's disease (PD) has met with little success. Mitochondrial dysfunction and elevated oxidative stress precede characteristic loss of dopamine-producing neurons from the midbrain in PD. The majority of PD cases are classified as sporadic (sPD) with an unknown etiology, whereas mutations in a handful of genes cause monogenic form called familial (fPD). Both sPD and fPD is characterized by protei...
Source: Molecular and Cellular Neuroscience - October 21, 2019 Category: Neuroscience Source Type: research

The mitochondria-targeted antioxidant MitoQ inhibits memory loss, neuropathology, and extends lifespan in aged 3xTg-AD mice
Publication date: Available online 12 September 2019Source: Molecular and Cellular NeuroscienceAuthor(s): Melissa L. Young, James L. FranklinAbstractOxidative stress, likely stemming from dysfunctional mitochondria, occurs before major cognitive deficits and neuropathologies become apparent in Alzheimer's disease (AD) patients and in mouse models of the disease. We previously reported that treating 2- to 7-month-old 3xTg-AD mice with the mitochondria-targeted antioxidant MitoQ (mitoquinone mesylate: [10-(4,5-Dimethoxy-2-methyl-3,6-dioxo-1,4-cyclohexadien-1-yl)decyl](triphenyl)phosphonium methanesulfonate), a period when AD...
Source: Molecular and Cellular Neuroscience - September 12, 2019 Category: Neuroscience Source Type: research

Editorial Board
Publication date: September 2019Source: Molecular and Cellular Neuroscience, Volume 99Author(s): (Source: Molecular and Cellular Neuroscience)
Source: Molecular and Cellular Neuroscience - September 10, 2019 Category: Neuroscience Source Type: research

Edaravone protects primary-cultured rat cortical neurons from ketamine-induced apoptosis via reducing oxidative stress and activating PI3K/Akt signal pathway
Publication date: Available online 7 September 2019Source: Molecular and Cellular NeuroscienceAuthor(s): Qianqian Li, Zhengguo Qiu, Yang Lu, Pan Lu, Jieqiong Wen, Kui Wang, Xijuan Zhao, Rong Li, Hong Zhang, Yan Zhang, Pengyu Jia, Pei Fan, Yuanyuan Zhang, Shuyue Zhang, Haixia Lv, Xinlin Chen, Yong Liu, Pengbo ZhangAbstractKetamine caused neuroapoptosis in the development of rat brain, in which oxidative stress play an important role. Edaravone (3-methyl-1-phenyl-2-pyrazolin-5-one), a free radical scavenger, exerts neuroprotective effects in many neurological disease models. Here we investigated whether edaravone protects pr...
Source: Molecular and Cellular Neuroscience - September 9, 2019 Category: Neuroscience Source Type: research