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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/28551798/mitochondrial-proton-leak-plays-a-critical-role-in-pathogenesis-of-cardiovascular-diseases
#2
Jiali Cheng, Gayani Nanayakkara, Ying Shao, Ramon Cueto, Luqiao Wang, William Y Yang, Ye Tian, Hong Wang, Xiaofeng Yang
Mitochondrial proton leak is the principal mechanism that incompletely couples substrate oxygen to ATP generation. This chapter briefly addresses the recent progress made in understanding the role of proton leak in the pathogenesis of cardiovascular diseases. Majority of the proton conductance is mediated by uncoupling proteins (UCPs) located in the mitochondrial inner membrane. It is evident that the proton leak and reactive oxygen species (ROS) generated from electron transport chain (ETC) in mitochondria are linked to each other...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28551015/salt-induced-phosphoproteomic-changes-in-the-hypothalamic-paraventricular-nucleus-in-rats-with-chronic-renal-failure
#3
Lanying Li, Jiawen Li, Lishan Tan, Minzi Qiu, Mengbi Zhang, Aiqing Li
Hypothalamic paraventricular nucleus (PVN) is a cardiovascular regulating center within the brain, which plays a critical role in high salt-induced progression of chronic renal failure (CRF). However, the phosphoproteomic changes in the PVN caused by CRF remain unclear. This study aimed to perform large-scale phosphoproteomic analysis of PVN induced by CRF and high salt intake. In this study, eight weeks post 5/6 nephrectomy (CRF model) or sham operation, Sprague-Dawley rats were fed a high-salt (4%) or normal-salt (0...
May 24, 2017: Brain Research
https://www.readbyqxmd.com/read/28550970/thoracotomy-in-the-emergency-department-for-resuscitation-of-the-mortally-injured
#4
J Christopher DiGiacomo, L D George Angus
PURPOSE: Emergency department resuscitative thoracotomy is an intervention of last resort for the acutely dying victim of trauma. In light of improvements in pre-hospital emergency systems, improved operative strategies for survival such as damage control and improvements in critical care medicine, the most extreme of resuscitation efforts should be re-evaluated for the potential survivor, with success properly defined as the return of vital signs which allow transport of the patient to the operating room...
May 10, 2017: Chinese Journal of Traumatology, Zhonghua Chuang Shang za Zhi
https://www.readbyqxmd.com/read/28550391/gut-liver-axis-cirrhosis-and-portal-hypertension-the-chicken-and-the-egg
#5
Juan P Arab, Rosa M Martin-Mateos, Vijay H Shah
The term gut-liver axis is used to highlight the close anatomical and functional relationship between the intestine and the liver. The intestine has a highly specialized epithelial membrane which regulates transport across the mucosa. Due to dysbiosis, impairment of the intestinal barrier and altered immunity status, bacterial products can reach the liver through the portal vein, where they are recognized by specific receptors, activate the immune system and lead to a proinflammatory response. Gut microbiota and bacterial translocation play an important role in the pathogenesis of chronic liver diseases, including alcoholic and non-alcoholic fatty liver disease, cirrhosis, and its complications, such as portal hypertension, spontaneous bacterial peritonitis and hepatic encephalopaty...
May 26, 2017: Hepatology International
https://www.readbyqxmd.com/read/28549825/metabolic-integration-of-bacterial-endosymbionts-through-antimicrobial-peptides
#6
REVIEW
Peter Mergaert, Yoshitomo Kikuchi, Shuji Shigenobu, Eva C M Nowack
Antimicrobial peptides (AMPs) are massively produced by eukaryotic hosts during symbiotic interactions with bacteria. Among other roles, these symbiotic AMPs have the capacity to permeabilize symbiont membranes and facilitate metabolite flow across the host-symbiont interface. We propose that an ancestral role of these peptides is to facilitate metabolic exchange between the symbiotic partners through membrane permeabilization. This function may be particularly critical for integration of endosymbiont and host metabolism in interactions involving bacteria with strongly reduced genomes lacking most small metabolite transporters...
May 23, 2017: Trends in Microbiology
https://www.readbyqxmd.com/read/28549591/single-allele-lmbrd1-knockout-results-in-cardiac-hypertrophy
#7
Linda Tzu-Ling Tseng, Chieh-Liang Lin, Kuei-Hsiang Pan, Kai-Yuan Tzen, Ming-Jai Su, Chia-Ti Tsai, Yi-Han Li, Pai-Chi Li, Fu-Tien Chiang, Shin C Chang, Ming-Fu Chang
BACKGROUND/PURPOSE: LMBD1 protein, a type IV-B plasma membrane protein possessing nine putative trans-membrane domains, was previously demonstrated at cellular level to play a critical part in the signaling cascade of insulin receptor through its involvement in regulating clathrin-mediated endocytosis. However, at physiological level, the significance of LMBD1 protein in cardiac development remains unclear. METHODS: To understand the role of Lmbrd1 gene involved in the cardiac function, heterozygous knockout mice were used as an animal model system...
May 23, 2017: Journal of the Formosan Medical Association, Taiwan Yi Zhi
https://www.readbyqxmd.com/read/28548087/the-distinct-metabolic-phenotype-of-lung-squamous-cell-carcinoma-defines-selective-vulnerability-to-glycolytic-inhibition
#8
Justin Goodwin, Michael L Neugent, Shin Yup Lee, Joshua H Choe, Hyunsung Choi, Dana M R Jenkins, Robin J Ruthenborg, Maddox W Robinson, Ji Yun Jeong, Masaki Wake, Hajime Abe, Norihiko Takeda, Hiroko Endo, Masahiro Inoue, Zhenyu Xuan, Hyuntae Yoo, Min Chen, Jung-Mo Ahn, John D Minna, Kristi L Helke, Pankaj K Singh, David B Shackelford, Jung-Whan Kim
Adenocarcinoma (ADC) and squamous cell carcinoma (SqCC) are the two predominant subtypes of non-small cell lung cancer (NSCLC) and are distinct in their histological, molecular and clinical presentation. However, metabolic signatures specific to individual NSCLC subtypes remain unknown. Here, we perform an integrative analysis of human NSCLC tumour samples, patient-derived xenografts, murine model of NSCLC, NSCLC cell lines and The Cancer Genome Atlas (TCGA) and reveal a markedly elevated expression of the GLUT1 glucose transporter in lung SqCC, which augments glucose uptake and glycolytic flux...
May 26, 2017: Nature Communications
https://www.readbyqxmd.com/read/28547793/the-anatomy-and-physiology-of-normal-and-abnormal-swallowing-in-oropharyngeal-dysphagia
#9
REVIEW
A Sasegbon, S Hamdy
BACKGROUND: Eating and drinking are enjoyable activities that positively impact on an individual's quality of life. The ability to swallow food and fluid is integral to the process of eating. Swallowing occupies a dual role being both part of the enjoyment of eating and being a critically important utilitarian activity to enable adequate nutrition and hydration. Any impairment to the process of swallowing can negatively affect a person's perception of their quality of life. The process of swallowing is highly complex and involves muscles in the mouth, pharynx, larynx, and esophagus...
May 25, 2017: Neurogastroenterology and Motility: the Official Journal of the European Gastrointestinal Motility Society
https://www.readbyqxmd.com/read/28547532/prehospital-blood-transfusions-in-pediatric-trauma-and-nontrauma-patients-a-single-center-review-of-safety-and-outcomes
#10
Aodhnait S Fahy, Cornelius A Thiels, Stephanie F Polites, Maile Parker, Michael B Ishitani, Christopher R Moir, Kathleen Berns, James R Stubbs, Donald H Jenkins, Scott P Zietlow, Martin D Zielinski
PURPOSE: Prehospital transfusions are a novel yet increasingly accepted intervention in the adult population as part of remote damage control resuscitation, but prehospital transfusions remain controversial in children. Our purpose was to review our pediatric prehospital transfusion experience over 12 years to describe the safety of prehospital transfusion in appropriately triaged trauma and nontrauma patients. METHODS: Children (<18 years) transfused with packed red blood cells (pRBC) or plasma during transport to a single regional academic medical center between 2002 and 2014 were identified...
May 25, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28546582/control-of-amino-acid-transport-coordinates-metabolic-reprogramming-in-t-cell-malignancy
#11
K M Grzes, M Swamy, J L Hukelmann, E Emslie, L V Sinclair, D A Cantrell
This study explores the regulation and importance of System L amino acid transport in a murine model of T cell acute lymphoblastic leukemia (T-ALL) caused by deletion of phosphatase and tensin homologue deleted on chromosome 10 (PTEN). There has been a strong focus on glucose transport in leukemias but the present data show that primary T-ALL cells have increased transport of multiple nutrients. Specifically, increased leucine transport in T-ALL fuels mammalian target of rapamycin complex 1 (mTORC1) activity which then sustains expression of hypoxia inducible factor-1α (HIF1α) and c-Myc; drivers of glucose metabolism in T cells...
May 26, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28545963/the-carboxy-terminal-region-of-the-tbc1d4-as160-rabgap-mediates-protein-homodimerization
#12
Ju Rang Woo, Soon-Jong Kim, Keon Young Kim, Hyonchol Jang, Steven E Shoelson, SangYoun Park
TBC1D4 (also known as AS160) is a Rab·GTPase-activating protein (RabGAP) which functions in insulin signaling. TBC1D4 is critical for translocation of glucose transporter 4 (GLUT4), from an inactive, intracellular, vesicle-bound site to the plasma membrane, where it promotes glucose entry into cells. The TBC1D4 protein is structurally subdivided into two N-terminal phosphotyrosine-binding (PTB) domains, a C-terminal catalytic RabGAP domain, and a disordered segment in between containing potential Akt phosphorylation sites...
May 22, 2017: International Journal of Biological Macromolecules
https://www.readbyqxmd.com/read/28545780/requirement-of-zinc-transporter-slc39a7-zip7-for-dermal-development-to-fine-tune-endoplasmic-reticulum-function-by-regulating-protein-disulfide-isomerase
#13
Bum-Ho Bin, Jinhyuk Bhin, Juyeon Seo, Se-Young Kim, Eunyoung Lee, Kyuhee Park, Dong-Hwa Choi, Teruhisa Takagishi, Takafumi Hara, Daehee Hwang, Haruhiko Koseki, Yoshinobu Asada, Shinji Shimoda, Kenji Mishima, Toshiyuki Fukada
Skin is the first area that manifests zinc deficiency. However, the molecular mechanisms by which zinc homeostasis affects skin development remain largely unknown. Here, we show that zinc-regulation transporter-/iron-regulation transporter-like protein 7 (ZIP7) localized to the endoplasmic reticulum plays critical roles in connective tissue development. Mice lacking the Slc39a7/Zip7 gene in collagen 1-expressing tissue exhibited dermal dysplasia. Ablation of ZIP7 in mesenchymal stem cells inhibited cell proliferation thereby preventing proper dermis formation, indicating that ZIP7 is required for dermal development...
May 17, 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28545156/the-boron-transporter-bnac4-bor1-1c-is-critical-for-inflorescence-development-and-fertility-under-boron-limitation-in-brassica-napus
#14
Quan Zhang, Haifei Chen, Mingliang He, Zhuqing Zhao, Hongmei Cai, Guangda Ding, Lei Shi, Fangsen Xu
Boron (B) is an essential micronutrient for plants, but the molecular mechanisms underlying the uptake and distribution of B in allotetraploid rapeseed (Brassica napus) are unclear. Here, we identified a B transporter of rapeseed, BnaC4.BOR1;1c, which expressed in shoot nodes and involved in distributing B to the reproductive organs. Transgenic Arabidopsis plants containing a BnaC4.BOR1;1c promoter-driven GUS reporter gene showed strong GUS activity in roots, nodal regions of the shoots and immature floral buds...
May 25, 2017: Plant, Cell & Environment
https://www.readbyqxmd.com/read/28544880/systems-wide-studies-uncover-commander-a-multiprotein-complex-essential-to-human-development
#15
REVIEW
Anna L Mallam, Edward M Marcotte
Recent mass spectrometry maps of the human interactome independently support the existence of a large multiprotein complex, dubbed "Commander." Broadly conserved across animals and ubiquitously expressed in nearly every human cell type examined thus far, Commander likely plays a fundamental cellular function, akin to other ubiquitous machines involved in expression, proteostasis, and trafficking. Experiments on individual subunits support roles in endosomal protein sorting, including the trafficking of Notch proteins, copper transporters, and lipoprotein receptors...
May 24, 2017: Cell Systems
https://www.readbyqxmd.com/read/28543042/the-ntcp-inhibitor-myrcludex-b-effects-on-bile-acid-disposition-and-tenofovir-pharmacokinetics
#16
Antje Blank, Annette Eidam, Mathias Haag, Nicolas Hohmann, Jürgen Burhenne, Matthias Schwab, Stan F J van de Graaf, Markus R Meyer, Hans H Maurer, Katrin Meier, Johanna Weiss, Thomas Bruckner, Alexander Alexandrov, Stephan Urban, Gerd Mikus, Walter E Haefeli
Myrcludex B acts as a hepatitis B and D virus entry inhibitor blocking the sodium taurocholate co-transporting polypeptide (SLC10A1). We investigated effects of myrcludex B on plasma bile acid disposition, tenofovir pharmacokinetics, and perpetrator characteristics on cytochrome P450 (CYP) 3A. Twelve healthy volunteers received 300 mg tenofovir disoproxil fumarate orally and 10 mg subcutaneous myrcludex B. Myrcludex B increased total plasma bile acid exposure 18.2-fold without signs of cholestasis. The rise in conjugated bile acids was up to 123-fold (taurocholic acid)...
May 24, 2017: Clinical Pharmacology and Therapeutics
https://www.readbyqxmd.com/read/28542554/sodium-tanshinone-iia-sulfonate-stimulated-cl-secretion-in-mouse-trachea
#17
Peng-Xiao Chen, Yi-Lin Zhang, Jia-Wen Xu, Ming-Hao Yu, Jie-Hong Huang, Lei Zhao, Wen-Liang Zhou
Sodium tanshinone IIA sulfonate (STS) is a derivate of tanshinone IIA, a lipophilic compound in Salvia miltiorrhiza. This study aimed to investigate the effect of STS on ion transport in mouse tracheal epithelium and the mechanisms underlying it. Short-circuit current (Isc) was measured to evaluate the effect of STS on transepithelial ion transport. Intracellular Ca2+ imaging was performed to observe intracellular Ca2+ concentration ([Ca2+]i) changes induced by STS in primary cultured mouse tracheal epithelial cells...
2017: PloS One
https://www.readbyqxmd.com/read/28542489/d-helix-influences-dimerization-of-the-atp-binding-cassette-abc-transporter-associated-with-antigen-processing-1-tap1-nucleotide-binding-domain
#18
Ahmet S Vakkasoglu, Sriram Srikant, Rachelle Gaudet
ATP-binding cassette (ABC) transporters form a large family of transmembrane importers and exporters. Using two nucleotide-binding domains (NBDs), which form a canonical ATP-sandwich dimer at some point within the transport cycle, the transporters harness the energy from ATP binding and hydrolysis to drive substrate transport. However the structural elements that enable and tune the dimerization propensity of the NBDs have not been fully elucidated. Here we compared the biochemical properties of the NBDs of human and rat TAP1, a subunit of the heterodimeric transporter associated with antigen processing (TAP)...
2017: PloS One
https://www.readbyqxmd.com/read/28542430/ros-regulation-of-axonal-mitochondrial-transport-is-mediated-by-ca2-and-jnk-in-drosophila
#19
Pin-Chao Liao, Lauren C Tandarich, Peter J Hollenbeck
Mitochondria perform critical functions including aerobic ATP production and calcium (Ca2+) homeostasis, but are also a major source of reactive oxygen species (ROS) production. To maintain cellular function and survival in neurons, mitochondria are transported along axons, and accumulate in regions with high demand for their functions. Oxidative stress and abnormal mitochondrial axonal transport are associated with neurodegenerative disorders. However, we know little about the connection between these two...
2017: PloS One
https://www.readbyqxmd.com/read/28540946/zinc-transporters-ybtx-and-znuabc-are-required-for-the-virulence-of-yersinia-pestis-in-bubonic-and-pneumonic-plague-in-mice
#20
Alexander G Bobrov, Olga Kirillina, Marina Y Fosso, Jacqueline D Fetherston, M Clarke Miller, Tiva T VanCleave, Joseph A Burlison, William K Arnold, Matthew B Lawrenz, Sylvie Garneau-Tsodikova, Robert D Perry
A number of bacterial pathogens require the ZnuABC Zinc (Zn(2+)) transporter and/or a second Zn(2+) transport system to overcome Zn(2+) sequestration by mammalian hosts. Previously we have shown that in addition to ZnuABC, Yersinia pestis possesses a second Zn(2+) transporter that involves components of the yersiniabactin (Ybt), siderophore-dependent iron transport system. Synthesis of the Ybt siderophore and YbtX, a member of the major facilitator superfamily, are both critical components of the second Zn(2+) transport system...
May 25, 2017: Metallomics: Integrated Biometal Science
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