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https://www.readbyqxmd.com/read/28551824/the-role-of-the-atp2c1-gene-in-hailey-hailey-disease
#1
REVIEW
Hao Deng, Heng Xiao
Hailey-Hailey disease (HHD) is a rare autosomal dominant acantholytic dermatosis, characterized by a chronic course of repeated and exacerbated skin lesions in friction regions. The pathogenic gene of HHD was reported to be the ATPase calcium-transporting type 2C member 1 gene (ATP2C1) located on chromosome 3q21-q24. Its function is to maintain normal intracellular concentrations of Ca(2+)/Mn(2+) by transporting Ca(2+)/Mn(2+) into the Golgi apparatus. ATP2C1 gene mutations are reportedly responsible for abnormal cytosolic Ca(2+)/Mn(2+) levels and the clinical manifestations of HHD...
May 27, 2017: Cellular and Molecular Life Sciences: CMLS
https://www.readbyqxmd.com/read/28550628/giovanni-verga-1879-1923-author-of-a-pioneering-treatise-on-pituitary-surgery-the-foundations-of-this-new-field-in-europe-in-the-early-1900s
#2
REVIEW
José M Pascual, Lorenzo Mongardi, Ruth Prieto, Inés Castro-Dufourny, María Rosdolsky, Sewan Strauss, Rodrigo Carrasco, Eduard Winter, Paolo Mazzarello
The field of pituitary surgery was born in the first decade of the twentieth century in Europe, and it evolved rapidly with the development of numerous innovative surgical techniques by some of the founding fathers of neurosurgery. This study investigates the pioneering Italian treatise on pituitary surgery, La Patologia Chirurgica dell'Ipofisi (Surgical Pathology of the Hypophysis), published in 1911 by Giovanni Verga (1879-1923), a surgeon from Pavía and one of Golgi's disciples. This little-known monograph compiles the earliest experience on pituitary surgery through the analysis of the first 50 procedures performed between 1903 and 1911...
May 27, 2017: Neurosurgical Review
https://www.readbyqxmd.com/read/28550530/rtms-ameliorates-ptsd-symptoms-in-rats-by-enhancing-glutamate-transmission-and-synaptic-plasticity-in-the-acc-via-the-pten-akt-signalling-pathway
#3
Gaohua Liu, Dayun Feng, Jian Wang, Haifeng Zhang, Zhengwu Peng, Min Cai, Jing Yang, Ruiguo Zhang, Huaning Wang, Shengxi Wu, Qingrong Tan
Repetitive transcranial magnetic stimulation (rTMS) is a novel physiological therapy that has been adopted to clinically treat psychiatric disorders. Our previous study indicated the potential therapeutic effect of rTMS on posttraumatic stress disorder (PTSD). However, the exact molecular mechanism is elusive. Currently, using the single prolonged stress (SPS) rat model for PTSD, we investigated the glutamatergic transmission and neural plasticity changes in the anterior cingulate cortex (ACC) after SPS induction and explored the protective effects and mechanism of rTMS treatment...
May 26, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28549691/27-hydroxycholesterol-upregulates-the-production-of-heat-shock-protein-60-of-monocytic-cells
#4
Bo-Young Kim, Yonghae Son, Jeongyoon Choi, Seong-Kug Eo, Young Chul Park, Koanhoi Kim
Investigating differentially expressed proteins in a milieu rich in cholesterol oxidation products, we found via mass spectrometry-based proteomics that surface levels of heat shock protein 60 (HSP60) were upregulated on monocytic cells in the presence of 27-hydroxycholesterol (27OHChol). The elevated levels of cytoplasmic membrane HSP60 were verified via Western blot analysis and visualized by confocal microscopy. Treatment with 27OHChol also resulted in increased levels of cellular HSP60 without altering its transcription...
May 23, 2017: Journal of Steroid Biochemistry and Molecular Biology
https://www.readbyqxmd.com/read/28549585/cystamine-mediated-inhibition-of-protein-disulfide-isomerase-triggers-aggregation-of-misfolded-orexin-a-in-the-golgi-apparatus-and-prevents-extracellular-secretion-of-orexin-a
#5
Issei Fujita, Mizuki Nobunaga, Takahiro Seki, Yuki Kurauchi, Akinori Hisatsune, Hiroshi Katsuki
Orexins (orexin-A and orexin-B) are neuropeptides that are reduced in narcolepsy, a sleep disorder that is characterized by excessive daytime sleepiness, sudden sleep attacks and cataplexy. However, it remains unclear how orexins in the brain and orexin neurons are reduced in narcolepsy. Orexin-A has two closely located intramolecular disulfide bonds and is prone to misfolding due to the formation of incorrect disulfide bonds. Protein disulfide isomerase (PDI) possesses disulfide interchange activity. PDI can modify misfolded orexin-A to its native form by rearrangement of two disulfide bonds...
May 23, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28549249/cd146-mcam-in-human-cs-dlk1-cs-cd34-adipose-stromal-progenitor-cells
#6
Susanne Hörl, Asim Ejaz, Sebastian Ernst, Monika Mattesich, Andreas Kaiser, Brigitte Jenewein, Marit E Zwierzina, Sarina Hammerle, Carina Miggitsch, Maria C Mitterberger-Vogt, Claudia Krautgasser, Gerhard Pierer, Werner Zwerschke
To precisely characterize CD146 in adipose stromal/progenitor cells (ASCs) we sorted the stromal vascular faction (SVF) of human abdominal subcutaneous white adipose tissue (sWAT) according to cell surface (cs) expression of CD146, DLK1 and CD34. This test identified three main SVF cell populations: ~50% cs-DLK1(-)/cs-CD34(+)/cs-CD146(-) ASCs, ~7.5% cs-DLK1(+)/cs-CD34(dim/+)/cs-CD146(+) and ~7.5% cs-DLK1(+)/cs-CD34(dim/+)/cs-CD146(-) cells. All cells contained intracellular CD146. Whole mount fluorescent IHC staining of small vessels detected CD146(+) endothelial cells (CD31(+)/CD34(+)/CD146(+)) and pericytes (CD31(-)/CD34(-)/CD146(+) ASCs)...
May 17, 2017: Stem Cell Research
https://www.readbyqxmd.com/read/28548701/microtubules-regulate-brush-border-formation
#7
Facundo M Tonucci, Anabela Ferretti, Evangelina Almada, Pamela Cribb, Rodrigo Vena, Florencia Hidalgo, Cristián Favre, Matt J Tyska, Irina Kaverina, M Cecilia Larocca
Most epithelial cells contain apical membrane structures associated to bundles of actin filaments, which constitute the brush border. Whereas microtubule participation in the maintenance of the brush border identity has been characterized, their contribution to de novo microvilli organization remained elusive. Hereby, using a cell model of individual enterocyte polarization, we found that nocodazole induced microtubule depolymerization prevented the de novo brush border formation. Microtubule participation in brush border actin organization was confirmed in polarized kidney tubule MDCK cells...
May 26, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28548464/control-of-cell-death-and-mitochondrial-fission-by-erk1-2-map-kinase-signalling
#8
REVIEW
Simon J Cook, Kate Stuart, Rebecca Gilley, Matthew J Sale
The ERK1/2 signalling pathway is best known for its role in connecting activated growth factor receptors to changes in gene expression due to activated ERK1/2 entering the nucleus and phosphorylating transcription factors. However, active ERK1/2 also translocate to a variety of other organelles including the endoplasmic reticulum, endosomes, golgi and mitochondria to access specific substrates and influence cell physiology. In this article we review two aspects of ERK1/2 signalling at the mitochondria that are involved in regulating cell fate decisions...
May 26, 2017: FEBS Journal
https://www.readbyqxmd.com/read/28547531/neonatal-hyperoxia-perturbs-neuronal-development-in-the-cerebellum
#9
Till Scheuer, Yuliya Sharkovska, Victor Tarabykin, Katharina Marggraf, Vivien Brockmöller, Christoph Bührer, Stefanie Endesfelder, Thomas Schmitz
Impaired postnatal brain development of preterm infants often results in neurological deficits. Besides pathologies of the forebrain, maldeveolopment of the cerebellum is increasingly recognized to contribute to psychomotor impairments of many former preterm infants. However, causes are poorly defined. We used a hyperoxia model to define neonatal damage in cerebellar granule cell precursors (GCPs) and in Purkinje cells (PCs) known to be essential for interaction with GCPs during development. We exposed newborn rats to 24 h 80% O2 from age P6 to P7 to identify postnatal and long-term damage in cerebellar GCPs at age P7 after hyperoxia and also after recovery in room air thereafter until P11 and P30...
May 25, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28547344/alteration-of-a-recombinant-protein-n-glycan-structure-in-silkworms-by-partial-suppression-of-n-acetylglucosaminidase-gene-expression
#10
Tatsuya Kato, Kotaro Kikuta, Ayumi Kanematsu, Sachiko Kondo, Hirokazu Yagi, Koichi Kato, Enoch Y Park
OBJECTIVE: To synthesize complex type N-glycans in silkworms, shRNAs against the fused lobe from Bombyx mori (BmFDL), which codes N-acetylglucosaminidase (GlcNAcase) in the Golgi, was expressed by recombinant B. mori nucleopolyhedrovirus (BmNPV) in silkworm larvae. RESULTS: Expression was under the control of the actin promoter of B. mori or the U6-2 and i.e.-2 promoters from Orgyia pseudotsugata multiple nucleopolyhedrovirus (OpMNPV). The reduction of specific GlcNAcase activity was observed in Bm5 cells and silkworm larvae using the U6-2 promoter...
May 25, 2017: Biotechnology Letters
https://www.readbyqxmd.com/read/28546447/signal-motifs-dependent-er-export-of-qc-snare-bet12-interacts-with-memb12-and-affects-pr1-trafficking-in-arabidopsis
#11
Kin Pan Chung, Yonglun Zeng, Yimin Li, Changyang Ji, Yiji Xia, Liwen Jiang
SNAREs are well-known for their role in controlling membrane fusion, the final but crucial step for vesicular transport in eukaryotes. SNARE proteins contribute to various biological processes including pathogen defense, channel activity regulation as well as plant growth and development. Precise targeting of SNARE proteins to destined compartments is a prerequisite for their proper functioning. However, the underlying mechanism(s) for SNAREs targeting in plants remains obscure. Here we investigate the targeting mechanism of the Qc-SNARE BET12, which is involved in protein trafficking in the early secretory pathway...
May 25, 2017: Journal of Cell Science
https://www.readbyqxmd.com/read/28546340/the-golgi-matrix-protein-giantin-is-required-for-normal-cilia-function-in-zebrafish
#12
Dylan J M Bergen, Nicola L Stevenson, Roderick E H Skinner, David J Stephens, Christina L Hammond
The Golgi is essential for glycosylation of newly synthesised proteins including almost all cell-surface and extracellular matrix proteoglycans. Giantin, encoded by the golgb1 gene, is a member of the golgin family of proteins that reside within the Golgi stack but its function remains elusive. Loss-of-function of giantin in rats causes osteochondrodysplasia; knockout mice show milder defects, notably a cleft palate. In vitro, giantin has been implicated in Golgi organisation, biosynthetic trafficking, and ciliogenesis...
May 25, 2017: Biology Open
https://www.readbyqxmd.com/read/28546289/a-tripeptidyl-peptidase-1-is-a-binding-partner-of-gphr-golgi-ph-regulator-in-dictyostelium
#13
Maria Stumpf, Rolf Müller, Berthold Gaßen, Regina Wehrstedt, Petra Fey, Malte A Karow, Ludwig Eichinger, Gernot Glöckner, Angelika A Noegel
Mutations in tripeptidyl peptidase 1 (TPP1) have been associated with late infantile neuronal ceroid lipofuscinosis (NCL2), a neurodegenerative disorder. TPP1 is a lysosomal serine protease, which removes tripeptides from the amino terminus of proteins and is composed of an N-terminal prodomain and a catalytic domain. It is conserved in mammals, amphibians, fish and the amoeba Dictyostelium discoideum. D. discoideum harbors at least six genes encoding tripeptidyl peptidase 1, tpp1A to tpp1F We identified TPP1F as binding partner of Dictyostelium GPHR (Golgi pH regulator), which is an evolutionary highly conserved intracellular transmembrane protein...
May 25, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28545181/hepatocellular-carcinoma-a-comprehensive-review-of-biomarkers-clinical-aspects-and-therapy
#14
Nathalia Martines Tunissiolli, Márcia Maria Urbanin Castanhole-Nunes, Patrícia Matos Biselli-Chicote, Érika Cristina Pavarino, Renato Ferreira da Silva, Rita de Cássia Martins Alves da Silva, Eny Maria Goloni-Bertollo
Hepatocellular carcinoma (HCC) is a cause of several deaths related to cancer worldwidely. In early stage, curative treatments such as surgical resection, liver transplant and local ablation can improve the patient ´s survival. However, the disease is detected in advanced stage; moreover some available therapies are restricted to palliative care and local treatment. Early detections of HCC and adequate therapy are crucial to increase survival as well as to improve the patient´s quality of life. Therefore, researchers have been investigating molecular biomarkers with high sensibility and reliability as Golgi 73 protein (GP73), Glypican-3 (GPC3), Osteopontin (OPN), microRNAs and others...
April 1, 2017: Asian Pacific Journal of Cancer Prevention: APJCP
https://www.readbyqxmd.com/read/28545157/the-aminoestrogen-prolame-increases-recognition-memory-and-hippocampal-neuronal-spine-density-in-aged-mice
#15
Alfonso Diaz, Samuel Treviño, Rubén Vázquez-Roque, Berenice Venegas, Blanca Espinosa, Gonzalo Flores, Juan Manuel Fernández-G, Luis F Montaño, Jorge Guevara
The aging brain shows biochemical and morphological changes in the dendrites of pyramidal neurons from the limbic system associated with memory loss. Prolame (N-(3-hydroxy-1,3,5 (10) -estratrien-17β-yl) -3-hydroxypropylamine) is a non-feminizing aminoestrogen with antithrombotic activity that prevents neuronal deterioration, oxidative stress and neuroinflammation. Our aim was to evaluate the effect of prolame on motor and cognitive processes, as well as its influence on the dendritic morphology of neurons at the CA1, CA3 and granule cells of the dentate gyrus (DG) regions of hippocampus (HP), and medium spiny neurons of the nucleus accumbens (NAcc) of aged mice...
May 25, 2017: Synapse
https://www.readbyqxmd.com/read/28543811/the-structural-flexibility-of-the-human-copper-chaperone-atox1-insights-from-combined-pulsed-epr-studies-and-computations
#16
Ariel R Levy, Meital Turgeman, Lada Gevorkyan-Aiapetov, Sharon Ruthstein
Metallochaperones are responsible for shuttling metal ions to target proteins. Thus, a metallochaperone's structure must be sufficiently flexible both to hold onto its ion while traversing the cytoplasm and to transfer the ion to or from a partner protein. Here, we sought to shed light on the structure of Atox1, a metallochaperone involved in the human copper regulation system. Atox1 shuttles copper ions from the main copper transporter, Ctr1, to the ATP7b transporter in the Golgi apparatus. Conventional biophysical tools such as X-ray or NMR cannot always target the various conformational states of metallochaperones, owing to a requirement for crystallography or low sensitivity and resolution...
May 20, 2017: Protein Science: a Publication of the Protein Society
https://www.readbyqxmd.com/read/28542518/rnf41-interacts-with-the-vps52-subunit-of-the-garp-and-earp-complexes
#17
Delphine Masschaele, Leentje De Ceuninck, Joris Wauman, Dieter Defever, Frank Stenner, Sam Lievens, Frank Peelman, Jan Tavernier
RNF41 (Ring Finger Protein 41) is an E3 ubiquitin ligase involved in the intracellular sorting and function of a diverse set of substrates. Next to BRUCE and Parkin, RNF41 can directly ubiquitinate ErbB3, IL-3, EPO and RARα receptors or downstream signaling molecules such as Myd88, TBK1 and USP8. In this way it can regulate receptor signaling and routing. To further elucidate the molecular mechanism behind the role of RNF41 in intracellular transport we performed an Array MAPPIT (Mammalian Protein-Protein Interaction Trap) screen using an extensive set of proteins derived from the human ORFeome collection...
2017: PloS One
https://www.readbyqxmd.com/read/28541511/molecular-association-of-arabidopsis-rth-with-its-homolog-rte1-in-regulating-ethylene-signaling
#18
Fangfang Zheng, Xiankui Cui, Maximo Rivarola, Ting Gao, Caren Chang, Chun-Hai Dong
The plant hormone ethylene affects many biological processes during plant growth and development. Ethylene is perceived by ethylene receptors at the endoplasmic reticulum (ER) membrane. The ETR1 ethylene receptor is positively regulated by the transmembrane protein RTE1, which localizes to the ER and Golgi apparatus. The RTE1 gene family is conserved in animals, plants, and lower eukaryotes. In Arabidopsis, RTE1-HOMOLOG (RTH) is the only homolog of the Arabidopsis RTE1 gene family. The regulatory function of the Arabidopsis RTH in ethylene signaling and plant growth is largely unknown...
May 24, 2017: Journal of Experimental Botany
https://www.readbyqxmd.com/read/28540373/in-vitro-cellular-behaviors-and-toxicity-assays-of-small-sized-fluorescent-silicon-nanoparticles
#19
Zhaohui Cao, Fei Peng, Zhilin Hu, Binbin Chu, Yiling Zhong, Yuanyuan Su, Sudan He, Yao He
Extensive investigations have been carried out for evaluating the toxicology of various nanomaterials (e.g., carbon- and metal-based nanomaterials), which offer invaluable information for assessing the feasibility of nanomaterial-based wide-ranging applications. In recent years, sufficient efforts have been made to develop fluorescent small-sized silicon nanoparticles (SiNPs) as a novel optical material simultaneously featuring strong fluorescence and ultrahigh photostability, providing high promise for a myriad of biological, biomedical and electronic applications...
May 25, 2017: Nanoscale
https://www.readbyqxmd.com/read/28540298/the-clinical-values-of-serum-markers-in-the-early-prediction-of-hepatocellular-carcinoma
#20
Bo Li, Boan Li, Tongsheng Guo, Zhiqiang Sun, Xiaohan Li, Xiaoxi Li, Han Wang, Weijiao Chen, Peng Chen, Yuanli Mao
The early prediction values of diagnostic markers for hepatocellular carcinoma (HCC) are still unclear at present. This study evaluated the prediction value of ten serum markers in HCC. A total of 109 cases of hepatic cirrhosis patients were followed up for 36 months and the relationship between the lifetime risk of developing HCC and levels of serum markers was analyzed. 31.2 (34/109) percent of hepatic cirrhosis patients developed HCC during the study's timeframe. Higher alpha-fetoprotein (AFP), alpha-fetoprotein-L3 (AFP-L3), alanine aminotransferase (ALT), and AFP-L3/AFP ratio levels are potential risk factors for malignization in hepatic cirrhosis patients (RR = 2...
2017: BioMed Research International
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