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Molecular Brain

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https://www.readbyqxmd.com/read/27903293/personalized-medicine-approach-confirms-a-milder-case-of-abat-deficiency
#1
A Besse, A K Petersen, J V Hunter, V Appadurai, S R Lalani, P E Bonnen
ABAT deficiency (OMIM 613163) is a rare inborn error of metabolism caused by recessive variants in the gene 4-aminobutyric acid transaminase (ABAT), which is responsible for both the catalysis of GABA and maintenance of nucleoside pools in the mitochondria. To date, only a few patients have been reported worldwide. Their clinical presentation has been remarkably consistent with primary features of severe psychomotor retardation, encephalopathy, hypotonia, and infantile-onset refractory epilepsy. We report a new case of ABAT deficiency that marks an important departure from previous clinical findings...
December 1, 2016: Molecular Brain
https://www.readbyqxmd.com/read/27809929/insight-of-brain-degenerative-protein-modifications-in-the-pathology-of-neurodegeneration-and-dementia-by-proteomic-profiling
#2
REVIEW
Sunil S Adav, Siu Kwan Sze
Dementia is a syndrome associated with a wide range of clinical features including progressive cognitive decline and patient inability to self-care. Due to rapidly increasing prevalence in aging society, dementia now confers a major economic, social, and healthcare burden throughout the world, and has therefore been identified as a public health priority by the World Health Organization. Previous studies have established dementia as a 'proteinopathy' caused by detrimental changes in brain protein structure and function that promote misfolding, aggregation, and deposition as insoluble amyloid plaques...
November 3, 2016: Molecular Brain
https://www.readbyqxmd.com/read/27770822/apcpeb4-a-non-prion-domain-containing-homolog-of-apcpeb-is-involved-in-the-initiation-of-long-term-facilitation
#3
Seung-Hee Lee, Jaehoon Shim, Ye-Hwang Cheong, Sun-Lim Choi, Yong-Woo Jun, Sue-Hyun Lee, Yeon-Su Chae, Jin-Hee Han, Yong-Seok Lee, Jin-A Lee, Chae-Seok Lim, Kausik Si, Stefan Kassabov, Igor Antonov, Eric R Kandel, Bong-Kiun Kaang, Deok-Jin Jang
Two pharmacologically distinct types of local protein synthesis are required for synapse- specific long-term synaptic facilitation (LTF) in Aplysia: one for initiation and the other for maintenance. ApCPEB, a rapamycin sensitive prion-like molecule regulates a form of local protein synthesis that is specifically required for the maintenance of the LTF. However, the molecular component of the local protein synthesis that is required for the initiation of LTF and that is sensitive to emetine is not known. Here, we identify a homolog of ApCPEB responsible for the initiation of LTF...
October 22, 2016: Molecular Brain
https://www.readbyqxmd.com/read/27760555/a-modulatory-role-of-asics-on-gabaergic-synapses-in-rat-hippocampal-cell-cultures
#4
Maksim Storozhuk, Elena Kondratskaya, Lyudmila Nikolaenko, Oleg Krishtal
Rapid acidification occurring during synaptic vesicle release can activate acid-sensing ion channels (ASICs) both on pre- and postsynaptic neurons. In the latter case, a fraction of postsynaptic current would be mediated by cation-selective acid-sensing ion channels. Additionally, in both cases, activation of acid-sensing ion channels could modulate synaptic strength by affecting transmitter release and/or sensitivity of postsynaptic receptors. To address potential involvement of acid-sensing ion channels in mediation/modulation of synaptic transmission at hippocampal GABAergic synapses, we studied effects of three structurally different blockers of acid-sensing ion channels on evoked postsynaptic currents using the patch-clamp technique...
October 19, 2016: Molecular Brain
https://www.readbyqxmd.com/read/27729083/disrupted-in-schizophrenia1-disc1-l100p-mutation-alters-synaptic-transmission-and-plasticity-in-the-hippocampus-and-causes-recognition-memory-deficits
#5
Lin Cui, Wei Sun, Ming Yu, Nan Li, Li Guo, Huating Gu, Yu Zhou
Disrupted-in-schizophrenia 1(DISC1) is a promising candidate susceptibility gene for a spectrum of psychiatric illnesses that share cognitive impairments in common, including schizophrenia, bipolar disorder and major depression. Here we report that DISC1 L100P homozygous mutant shows normal anxiety- and depression-like behavior, but impaired object recognition which is prevented by administration of atypical antipsychotic drug clozapine. Ca(2+) image analysis reveals suppression of glutamate-evoked elevation of cytoplasmic [Ca(2+)] in L100P hippocampal slices...
October 12, 2016: Molecular Brain
https://www.readbyqxmd.com/read/27716287/modeling-neurological-diseases-with-induced-pluripotent-cells-reprogrammed-from-immortalized-lymphoblastoid-cell-lines
#6
Koki Fujimori, Toshiki Tezuka, Hiroyuki Ishiura, Jun Mitsui, Koichiro Doi, Jun Yoshimura, Hirobumi Tada, Takuya Matsumoto, Miho Isoda, Ryota Hashimoto, Nubutaka Hattori, Takuya Takahashi, Shinichi Morishita, Shoji Tsuji, Wado Akamatsu, Hideyuki Okano
Patient-specific induced pluripotent stem cells (iPSCs) facilitate understanding of the etiology of diseases, discovery of new drugs and development of novel therapeutic interventions. A frequently used starting source of cells for generating iPSCs has been dermal fibroblasts (DFs) isolated from skin biopsies. However, there are also numerous repositories containing lymphoblastoid B-cell lines (LCLs) generated from a variety of patients. To date, this rich bioresource of LCLs has been underused for generating iPSCs, and its use would greatly expand the range of targeted diseases that could be studied by using patient-specific iPSCs...
October 3, 2016: Molecular Brain
https://www.readbyqxmd.com/read/27716400/perk-regulates-gq-protein-coupled-intracellular-ca-2-dynamics-in-primary-cortical-neurons
#7
Siying Zhu, Barbara C McGrath, Yuting Bai, Xin Tang, Douglas R Cavener
PERK (EIF2AK3) is an ER-resident eIF2α kinase required for behavioral flexibility and metabotropic glutamate receptor-dependent long-term depression via its translational control. Motivated by the recent discoveries that PERK regulates Ca(2+) dynamics in insulin-secreting β-cells underlying glucose-stimulated insulin secretion, and modulates Ca(2+) signals-dependent working memory, we explored the role of PERK in regulating Gq protein-coupled Ca(2+) dynamics in pyramidal neurons. We found that acute PERK inhibition by the use of a highly specific PERK inhibitor reduced the intracellular Ca(2+) rise stimulated by the activation of acetylcholine, metabotropic glutamate and bradykinin-2 receptors in primary cortical neurons...
October 1, 2016: Molecular Brain
https://www.readbyqxmd.com/read/27680101/in-vivo-whole-cell-recording-with-high-success-rate-in-anaesthetized-and-awake-mammalian-brains
#8
Yao Wang, Yu-Zhang Liu, Shi-Yi Wang, Zhiru Wang
As a critical technique for dissection of synaptic and cellular mechanisms, whole-cell patch-clamp recording has become feasible for in vivo preparations including both anaesthetized and awake mammalian brains. However, compared with in vitro whole-cell recording, in vivo whole-cell recording often suffers from low success rates and high access resistance, preventing its wide application in physiological analysis of neural circuits. Here, we describe experimental procedures for achieving in vivo amphotericin B-perforated whole-cell recording as well as conventional (breakthrough) whole-cell recording from rats and mice...
September 29, 2016: Molecular Brain
https://www.readbyqxmd.com/read/27234425/calcium-activated-adenylyl-cyclase-ac8-but-not-ac1-is-required-for-prolonged-behavioral-anxiety
#9
Matteo Bernabucci, Min Zhuo
BACKGROUND: Anxiety disorder is a state of mental discomfort while acute anxiety induces an enhancement of vigilance/arousal or increased anxious responses. Most of the previous studies investigated basic mechanisms for acute anxiety, while less information is available for prolonged or repetitive anxiety. RESULTS: In the present study, we wanted to examine possible molecular mechanisms for behavioral anxiety after repeated exposures. Performing a paradigm of five sessions of the elevated plus-maze (EPM), we show that the repeated exposure to the EPM induces a long-lasting anxiety causing a gradual increase of anxiolytic activity, which is maintained for at least 21 days...
May 27, 2016: Molecular Brain
https://www.readbyqxmd.com/read/27107575/lncrna-nonratt021972-sirna-regulates-neuropathic-pain-behaviors-in-type-2-diabetic-rats-through-the-p2x7-receptor-in-dorsal-root-ganglia
#10
Shuangmei Liu, Lifang Zou, Jinyan Xie, Wei Xie, Shiyao Wen, Qiuyu Xie, Yun Gao, Guilin Li, Chunping Zhang, Changshui Xu, Hong Xu, Bing Wu, Qiulan Lv, Xi Zhang, Shouyu Wang, Yun Xue, Shangdong Liang
BACKGROUND: Long non-protein-coding RNAs (lncRNAs) are involved in the pathological processes of nervous system diseases. NONRATT021972 is an lncRNA. This study explores the effects of lncRNA NONRATT021972 small interference RNA (siRNA) on diabetic neuropathic pain (DNP) mediated by the P2X7 receptor in the rat dorsal root ganglia (DRG). RESULTS: Our results show that NONRATT021972 expression was significantly higher in the DRG of diabetes mellitus (DM) group compared with control group...
April 23, 2016: Molecular Brain
https://www.readbyqxmd.com/read/27090516/linking-f-box-protein-7-and-parkin-to-neuronal-degeneration-in-parkinson-s-disease-pd
#11
REVIEW
Zhi Dong Zhou, Sushmitha Sathiyamoorthy, Dario C Angeles, Eng King Tan
Mutations of F-box protein 7 (FBXO7) and Parkin, two proteins in ubiquitin-proteasome system (UPS), are both implicated in pathogenesis of dopamine (DA) neuron degeneration in Parkinson's disease (PD). Parkin is a HECT/RING hybrid ligase that physically receives ubiquitin on its catalytic centre and passes ubiquitin onto its substrates, whereas FBXO7 is an adaptor protein in Skp-Cullin-F-box (SCF) SCF(FBXO7) ubiquitin E3 ligase complex to recognize substrates and mediate substrates ubiquitination by SCF(FBXO7) E3 ligase...
April 18, 2016: Molecular Brain
https://www.readbyqxmd.com/read/27080240/reductions-in-hypothalamic-gfap-expression-glial-cells-and-%C3%AE-tanycytes-in-lean-and-hypermetabolic-gnasxl-deficient-mice
#12
Andrew P Holmes, Shi Quan Wong, Michela Pulix, Kirsty Johnson, Niamh S Horton, Patricia Thomas, João Pedro de Magalhães, Antonius Plagge
BACKGROUND: Neuronal and glial differentiation in the murine hypothalamus is not complete at birth, but continues over the first two weeks postnatally. Nutritional status and Leptin deficiency can influence the maturation of neuronal projections and glial patterns, and hypothalamic gliosis occurs in mouse models of obesity. Gnasxl constitutes an alternative transcript of the genomically imprinted Gnas locus and encodes a variant of the signalling protein Gαs, termed XLαs, which is expressed in defined areas of the hypothalamus...
April 14, 2016: Molecular Brain
https://www.readbyqxmd.com/read/27004687/regulation-of-lrrk2-promoter-activity-and-gene-expression-by-sp1
#13
Juelu Wang, Weihong Song
BACKGROUND: The dopaminergic neurodegeneration in the nigrostriatal pathway is a prominent neuropathological feature of Parkinson's disease (PD). Mutations in various genes have been linked to familial PD, and leucine-rich repeat kinase 2 (LRRK2) gene is one of them. LRRK2 is a large complex protein, belonging to the ROCO family of proteins. Recent studies suggest that the level of LRRK2 protein is one of the contributing factors to PD pathogenesis. However, it remains elusive how LRRK2 is regulated at the transcriptional and translational level...
March 22, 2016: Molecular Brain
https://www.readbyqxmd.com/read/27000207/feedback-modulation-of-neural-network-synchrony-and-seizure-susceptibility-by-mdm2-p53-nedd4-2-signaling
#14
Kathryn A Jewett, Catherine A Christian, Jonathan T Bacos, Kwan Young Lee, Jiuhe Zhu, Nien-Pei Tsai
BACKGROUND: Neural network synchrony is a critical factor in regulating information transmission through the nervous system. Improperly regulated neural network synchrony is implicated in pathophysiological conditions such as epilepsy. Despite the awareness of its importance, the molecular signaling underlying the regulation of neural network synchrony, especially after stimulation, remains largely unknown. RESULTS: In this study, we show that elevation of neuronal activity by the GABA(A) receptor antagonist, Picrotoxin, increases neural network synchrony in primary mouse cortical neuron cultures...
March 22, 2016: Molecular Brain
https://www.readbyqxmd.com/read/26928278/specific-cytoarchitectureal-changes-in-hippocampal-subareas-in-dadream-mice
#15
Britt Mellström, Asta Kastanauskaite, Shira Knafo, Paz Gonzalez, Xose M Dopazo, Ana Ruiz-Nuño, John G R Jefferys, Min Zhuo, Tim V P Bliss, Jose R Naranjo, Javier DeFelipe
BACKGROUND: Transcriptional repressor DREAM (downstream regulatory element antagonist modulator) is a Ca(2+)-binding protein that regulates Ca(2+) homeostasis through gene regulation and protein-protein interactions. It has been shown that a dominant active form (daDREAM) is implicated in learning-related synaptic plasticity such as LTP and LTD in the hippocampus. Neuronal spines are reported to play important roles in plasticity and memory. However, the possible role of DREAM in spine plasticity has not been reported...
February 29, 2016: Molecular Brain
https://www.readbyqxmd.com/read/26892330/dementia-linked-amyloidosis-is-associated-with-brain-protein-deamidation-as-revealed-by-proteomic-profiling-of-human-brain-tissues
#16
Sunil S Adav, Xavier Gallart-Palau, Kok Hian Tan, Sai Kiang Lim, James P Tam, Siu Kwan Sze
BACKGROUND: Aggregation of malformed proteins is a key feature of many neurodegenerative diseases, but the mechanisms that drive proteinopathy in the brain are poorly understood. We aimed to characterize aggregated proteins in human brain tissues affected by dementia. RESULTS: To characterize amyloidal plaque purified from post-mortem brain tissue of dementia patient, we applied ultracentrifugation-electrostatic repulsion hydrophilic interaction chromatography (UC-ERLIC) coupled mass spectrometry-based proteomics technologies...
February 19, 2016: Molecular Brain
https://www.readbyqxmd.com/read/26879639/rab18-a-protein-associated-with-warburg-micro-syndrome-controls-neuronal-migration-in-the-developing-cerebral-cortex
#17
Qinwei Wu, Xiaqin Sun, Weihua Yue, Tianlan Lu, Yanyan Ruan, Tianda Chen, Dai Zhang
BACKGROUND: Loss of function mutations in RAB18, has been identified in patients with the human neurological and developmental disorder Warburg Micro syndrome. However, the function of RAB18 in brain remains unknown. RESULTS: In this study, we report that RAB18 is a critical regulator of neuronal migration and morphogenesis. Using in utero electroporation suppression of RAB18 in the mouse brain impairs radial migration. Overexpression of dominant negative RAB18 or disruption of RAB3GAP (RAB18GEF) also results in delayed neuronal migration in the developing mouse cortex and inhibition of neurite growth in vitro...
February 16, 2016: Molecular Brain
https://www.readbyqxmd.com/read/26876533/maternal-sleep-deprivation-at-different-stages-of-pregnancy-impairs-the-emotional-and-cognitive-functions-and-suppresses-hippocampal-long-term-potentiation-in-the-offspring-rats
#18
Yan Peng, Wei Wang, Tao Tan, Wenting He, Zhifang Dong, Yu Tian Wang, Huili Han
BACKGROUND: Sleep deprivation during pregnancy is a serious public health problem as it can affect the health of pregnant women and newborns. However, it is not well studied whether sleep deprivation at different stages of pregnancy has similar effects on emotional and cognitive functions of the offspring, and if so, the potential cellular mechanisms also remain poorly understood. METHODS: In the present study, the pregnant rats were subjected to sleep deprivation for 6 h per day by gentle handling during the first (gestational days 1-7), second (gestational days 8-14) and third trimester (gestational days 15-21) of pregnancy, respectively...
February 15, 2016: Molecular Brain
https://www.readbyqxmd.com/read/26856818/comprehensive-behavioral-phenotyping-of-a-new-semaphorin-3%C3%A2-f-mutant-mouse
#19
Ikuo Matsuda, Hirotaka Shoji, Nobuyuki Yamasaki, Tsuyoshi Miyakawa, Atsu Aiba
BACKGROUND: Semaphorin 3 F (Sema3F) is a secreted type of the Semaphorin family of axon guidance molecules. Sema3F and its receptor neuropilin-2 (Npn-2) are expressed in a mutually exclusive manner in the embryonic mouse brain regions including olfactory bulb, hippocampus, and cerebral cortex. Sema3F is thought to have physiological functions in the formation of neuronal circuitry and its refinement. However, functional roles of Sema3F in the brain remain to be clarified. Here, we examined behavioral effects of Sema3F deficiency through a comprehensive behavioral test battery in Sema3F knockout (KO) male mice to understand the possible functions of Sema3F in the brain...
February 9, 2016: Molecular Brain
https://www.readbyqxmd.com/read/26856703/impaired-learning-and-memory-in-cd38-null-mutant-mice
#20
Somi Kim, TaeHyun Kim, Hye-Ryeon Lee, Eun-Hye Jang, Hyun-Hee Ryu, Minkyung Kang, So-Young Rah, Juyoun Yoo, Bolam Lee, Jae-Ick Kim, Chae Seok Lim, Sang Jeong Kim, Uh-Hyun Kim, Yong-Seok Lee, Bong-Kiun Kaang
CD38 is an enzyme that catalyzes the formation of cyclic ADP ribose and nicotinic acid adenine dinucleotide phosphate, both of which are involved in the mobilization of Ca(2+) from intracellular stores. Recently, CD38 has been shown to regulate oxytocin release from hypothalamic neurons. Importantly, CD38 mutations are associated with autism spectrum disorders (ASD) and CD38 knockout (CD38(-/-)) mice display ASD-like behavioral phenotypes including deficient parental behavior and poor social recognition memory...
February 9, 2016: Molecular Brain
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