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https://www.readbyqxmd.com/read/28219397/immunohistochemistry-of-sarcolemmal-membrane-associated-proteins-in-formalin-fixed-and-paraffin-embedded-skeletal-muscle-tissue-a-promising-tool-for-the-diagnostic-evaluation-of-common-muscular-dystrophies
#1
Chinnawut Suriyonplengsaeng, Charungthai Dejthevaporn, Chaiyos Khongkhatithum, Suda Sanpapant, Nattha Tubthong, Koset Pinpradap, Nippa Srinark, Jariya Waisayarat
BACKGROUND: The analysis of fresh frozen muscle specimens is standard following routine muscle biopsy, but this service is not widely available in countries with limited medical facilities, such as Thailand. Nevertheless, immunohistochemistry (IHC) analysis is essential for the diagnosis of patients with a strong clinical suspicion of muscular dystrophy, in the absence of mutations detected by molecular genetics. As the successful labelling of sarcolemmal membrane-associated proteins in formalin-fixed and paraffin-embedded (FFPE) muscle sections using IHC staining has rarely been described, this study aimed to develop a reproducible IHC method for such an analysis...
February 20, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28193846/protein-4-1r-exon-16-3-splice-site-activation-requires-coordination-among-tia1-pcbp1-and-rbm39-during-terminal-erythropoiesis
#2
Shu-Ching Huang, Henry S Zhang, Brian Yu, Ellen McMahon, Dan T Nguyen, Faye H Yu, Alexander C Ou, Jennie Park Ou, Edward J Benz
Exon 16 of protein 4.1R encodes a spectrin/actin-binding peptide critical for erythrocyte membrane stability. Its expression during erythroid differentiation is regulated by alternative pre-mRNA splicing. A UUUUCCCCCC-motif situated between the branch point and the 3' splice site is crucial for inclusion. We show that the "UUUU" region and the last 3 C's in this motif are necessary for the binding of splicing factors TIA1 and Pcbp1, and that these proteins appear to act in a collaborative manner to enhance exon 16 inclusion...
February 13, 2017: Molecular and Cellular Biology
https://www.readbyqxmd.com/read/28190474/salvianolic-acid-a-inhibits-calpain-activation-and-enos-uncoupling-during-focal-cerebral-ischemia-in-mice
#3
Qaisar Mahmood, Guang-Fa Wang, Gang Wu, Huan Wang, Chang-Xin Zhou, Hong-Yu Yang, Zhi-Rong Liu, Feng Han, Kui Zhao
BACKGROUND: Salvianolic acid A (SAA) is obtained from Chinese herb Salviae Miltiorrhizae Bunge (Labiatae), has been reported to have the protective effects against cardiovascular and neurovascular diseases. HYPOTHESIS: The aim of present study was to investigate the relationship between the effectiveness of SAA against neurovascular injury and its effects on calpain activation and endothelial nitric oxide synthase (eNOS) uncoupling. STUDY DESIGN: SAA or vehicle was given to C57BL/6 male mice for seven days before the occlusion of middle cerebral artery (MCAO) for 60min...
February 15, 2017: Phytomedicine: International Journal of Phytotherapy and Phytopharmacology
https://www.readbyqxmd.com/read/28179572/spectrin-%C3%AE-v-adaptive-mutations-and-changes-in-subcellular-location-correlate-with-emergence-of-hair-cell-electromotility-in-mammalians
#4
Matteo Cortese, Samantha Papal, Francisco Pisciottano, Ana Belén Elgoyhen, Jean-Pierre Hardelin, Christine Petit, Lucia Florencia Franchini, Aziz El-Amraoui
The remarkable hearing capacities of mammals arise from various evolutionary innovations. These include the cochlear outer hair cells and their singular feature, somatic electromotility, i.e., the ability of their cylindrical cell body to shorten and elongate upon cell depolarization and hyperpolarization, respectively. To shed light on the processes underlying the emergence of electromotility, we focused on the βV giant spectrin, a major component of the outer hair cells' cortical cytoskeleton. We identified strong signatures of adaptive evolution at multiple sites along the spectrin-βV amino acid sequence in the lineage leading to mammals, together with substantial differences in the subcellular location of this protein between the frog and the mouse inner ear hair cells...
February 8, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28173092/posterior-cerebellar-purkinje-cells-in-an-sca5-sparca1-mouse-model-are-especially-vulnerable-to-the-synergistic-effect-of-loss-of-%C3%AE-iii-spectrin-and-glast
#5
Emma M Perkins, Daumante Suminaite, Yvonne L Clarkson, Sin Kwan Lee, Alastair R Lyndon, Jeffrey D Rothstein, David J A Wyllie, Kohichi Tanaka, Mandy Jackson
No abstract text is available yet for this article.
October 15, 2016: Human Molecular Genetics
https://www.readbyqxmd.com/read/28163384/association-between-alcohol-induced-erythrocyte-membrane-alterations-and-hemolysis-in-chronic-alcoholics
#6
Saradamma Bulle, Vaddi Damodara Reddy, Pannuru Padmavathi, Paramahamsa Maturu, Pavan Kumar Puvvada, Varadacharyulu Nallanchakravarthula
The present study aimed to understand the association between erythrocyte membrane alterations and hemolysis in chronic alcoholics. Study was conducted on human male volunteers aged between 35-45 years with a drinking history of 8-10 years. Results showed that plasma marker enzymes AST, ALT, ALP and γGT were increased in alcoholic subjects. Plasma and erythrocyte membrane lipid peroxidation, erythrocyte lysate nitric oxide (NOx) levels were also increased significantly in alcoholics. Furthermore, erythrocyte membrane protein carbonyls, total cholesterol, phospholipid and cholesterol/phospholipid (C/P) ratio were increased in alcoholics...
January 2017: Journal of Clinical Biochemistry and Nutrition
https://www.readbyqxmd.com/read/28150589/effects-of-heat-and-freeze-on-isolated-erythrocyte-submembrane-skeletons
#7
Ivan T Ivanov, Boyana K Paarvanova, Veselin Ivanov, Kathrin Smuda, Hans Bäumler, Radostina Georgieva
In this study we heated insoluble residues, obtained after Triton-X-100 (0.1 v/v%) extraction of erythrocyte ghost membranes (EGMs). Specific heat capacity, electric capacitance and resistance, and optical transmittance (280 nm) sustained sharp changes at 49°C (TA) and 66°C (TC), the known denaturation temperatures of spectrin and band 3, respectively. The change at TA was selectively inhibited by diamide (1 mM) and taurine mustard (1 mM) while its inducing temperature was selectively decreased by formamide in full concert with the assumed involvement of spectrin denaturation...
February 2, 2017: General Physiology and Biophysics
https://www.readbyqxmd.com/read/28148727/early-and-late-loss-of-the-cytoskeletal-scaffolding-protein-ankyrin-g-reveals-its-role-in-maturation-and-maintenance-of-nodes-of-ranvier-in-myelinated-axons
#8
Julia Saifetiarova, Anna M Taylor, Manzoor A Bhat
: The mechanisms that govern node of Ranvier organization, stability and long-term maintenance remain to be fully elucidated. One of the molecular components of the node is the cytoskeletal scaffolding protein, Ankyrin G (AnkG), which interacts with multiple members of the nodal complex. The role of AnkG in nodal organization and maintenance is still not clearly defined, as to whether AnkG functions as an initial nodal organizer or whether it functions as a nodal stabilizer after the nodal complex has been assembled...
February 1, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28143451/distinct-prostate-cancer-related-mrna-cargo-in-extracellular-vesicle-subsets-from-prostate-cell-lines
#9
Elisa Lázaro-Ibáñez, Taral R Lunavat, Su Chul Jang, Carmen Escobedo-Lucea, Jorge Oliver-De La Cruz, Pia Siljander, Jan Lötvall, Marjo Yliperttula
BACKGROUND: Multiple types of extracellular vesicles (EVs), including microvesicles (MVs) and exosomes (EXOs), are released by all cells constituting part of the cellular EV secretome. The bioactive cargo of EVs can be shuffled between cells and consists of lipids, metabolites, proteins, and nucleic acids, including multiple RNA species from non-coding RNAs to messenger RNAs (mRNAs). In this study, we hypothesized that the mRNA cargo of EVs could differ based on the EV cellular origin and subpopulation analyzed...
February 1, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28134616/the-paranodal-cytoskeleton-clusters-na-channels-at-nodes-of-ranvier
#10
Veronique Amor, Chuansheng Zhang, Anna Vainshtein, Ao Zhang, Daniel R Zollinger, Yael Eshed-Eisenbach, Peter J Brophy, Matthew N Rasband, Elior Peles
A high density of Na(+) channels at nodes of Ranvier is necessary for rapid and efficient action potential propagation in myelinated axons. Na+ channel clustering is thought to depend on two axonal cell adhesion molecules that mediate interactions between the axon and myelinating glia at the nodal gap (i.e., NF186) and the paranodal junction (i.e., Caspr). Here we show that while Na(+) channels cluster at nodes in the absence of NF186, they fail to do so in double conditional knockout mice lacking both NF186 and the paranodal cell adhesion molecule Caspr, demonstrating that a paranodal junction-dependent mechanism can cluster Na(+) channels at nodes...
January 30, 2017: ELife
https://www.readbyqxmd.com/read/28130160/chelerythrine-promotes-ca-2-dependent-calpain-activation-in-neuronal-cells-in-a-pkc-independent-manner
#11
Ana Saavedra, Sara Fernández-García, Silvia Cases, Mar Puigdellívol, Rafael Alcalá-Vida, Núria Martín-Flores, Jordi Alberch, Silvia Ginés, Cristina Malagelada, Esther Pérez-Navarro
BACKGROUND: Chelerythrine is widely used as a broad range protein kinase C (PKC) inhibitor, but there is controversy about its inhibitory effect. Moreover, it has been shown to exert PKC-independent effects on non-neuronal cells. METHODS: In this study we investigated possible off-target effects of chelerythrine on cultured cortical rodent neurons and a neuronal cell line. RESULTS: We found that 10μM chelerythrine, a commonly used concentration in neuronal cultures, reduces PKC and cAMP-dependent protein kinase substrates phosphorylation in mouse cultured cortical neurons, but not in rat primary cortical neurons or in a striatal cell line...
January 24, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28123494/sbdps-and-tau-proteins-for-diagnosis-and-hypothermia-therapy-in-neonatal-hypoxic-ischemic-encephalopathy
#12
Hongwei Wu, Zhenguang Li, Xia Yang, Jinfeng Liu, Wei Wang, Gang Liu
The use of spectrin breakdown products (SBDPs) and Tau protein levels for diagnosis and a mild hypothermia therapy for treatment of neonatal hypoxic-ischemic encephalopathy (HIE) was evaluated. One hundred and fifty infants, with HIE within 12 h after birth, participated in the study. There were 30 newborns with mild symptoms, 60 with moderate symptoms, 60 with severe symptoms, and 30 in a control group. Regular therapy was used for the control and the mild HIE groups, and also for 30 cases in the group with moderate symptoms and for 30 in the group with severe symptoms...
January 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28114741/loss-of-the-transforming-growth-factor-%C3%AE-effector-%C3%AE-2-spectrin-promotes-genomic-instability
#13
Jian Chen, Vivek Shukla, Patrizia Farci, Jaclyn Andricovich, Wilma Jogunoori, Lawrence N Kwong, Lior H Katz, Kirti Shetty, Asif Rashid, Xiaoping Su, Jon White, Lei Li, Alan Yaoqi Wang, Boris Blechacz, Gottumukkala S Raju, Marta Davila, Bao-Ngoc Nguyen, John R Stroehlein, Junjie Chen, Sang Soo Kim, Heather Levin, Keigo Machida, Hidekazu Tsukamoto, Peter Michaely, Alexandros Tzatsos, Bibhuti Mishra, Richard Amdur, Lopa Mishra
: Exposure to genotoxins such as ethanol-derived acetaldehyde leads to DNA damage and liver injury and promotes the development of cancer. We report here a major role for the transforming growth factor β/mothers against decapentaplegic homolog 3 adaptor β2-Spectrin (β2SP, gene Sptbn1) in maintaining genomic stability following alcohol-induced DNA damage. β2SP supports DNA repair through β2SP-dependent activation of Fanconi anemia complementation group D2 (Fancd2), a core component of the Fanconi anemia complex...
February 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28112730/cotranslational-folding-of-spectrin-domains-via-partially-structured-states
#14
Ola B Nilsson, Adrian A Nickson, Jeffrey J Hollins, Stephan Wickles, Annette Steward, Roland Beckmann, Gunnar von Heijne, Jane Clarke
How do the key features of protein folding, elucidated from studies on native, isolated proteins, manifest in cotranslational folding on the ribosome? Using a well-characterized family of homologous α-helical proteins with a range of biophysical properties, we show that spectrin domains can fold vectorially on the ribosome and may do so via a pathway different from that of the isolated domain. We use cryo-EM to reveal a folded or partially folded structure, formed in the vestibule of the ribosome. Our results reveal that it is not possible to predict which domains will fold within the ribosome on the basis of the folding behavior of isolated domains; instead, we propose that a complex balance of the rate of folding, the rate of translation and the lifetime of folded or partly folded states will determine whether folding occurs cotranslationally on actively translating ribosomes...
January 23, 2017: Nature Structural & Molecular Biology
https://www.readbyqxmd.com/read/28112201/novel-bioinformatics-based-approach-for-proteomic-biomarkers-prediction-of-calpain-2-caspase-3-protease-fragmentation-application-to-%C3%AE-ii-spectrin-protein
#15
Atlal El-Assaad, Zaher Dawy, Georges Nemer, Firas Kobeissy
The crucial biological role of proteases has been visible with the development of degradomics discipline involved in the determination of the proteases/substrates resulting in breakdown-products (BDPs) that can be utilized as putative biomarkers associated with different biological-clinical significance. In the field of cancer biology, matrix metalloproteinases (MMPs) have shown to result in MMPs-generated protein BDPs that are indicative of malignant growth in cancer, while in the field of neural injury, calpain-2 and caspase-3 proteases generate BDPs fragments that are indicative of different neural cell death mechanisms in different injury scenarios...
January 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28112189/the-potential-role-of-spectrin-network-in-the-mechanotransduction-of-mlo-y4-osteocytes
#16
Xin-Tong Wu, Lian-Wen Sun, Xiao Yang, Dong Ding, Dong Han, Yu-Bo Fan
The spectrin is first identified as the main component of erythrocyte membrane skeleton. It is getting growing attention since being found in multiple nonerythroid cells, providing complex mechanical properties and signal interface under the cell membrane. Recent genomics studies have revealed that the spectrin is highly relevant to bone disorders. However, in osteocytes, the important mechanosensors in bone, the role of spectrin is poorly understood. In this research, the role of spectrin in the mechanotransduction of MLO-Y4 osteocytes was studied...
January 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28101868/dystrophin-and-spectrin-two-highly-dissimilar-sisters-of-the-same-family
#17
Olivier Delalande, Aleksander Czogalla, Jean-François Hubert, Aleksander Sikorski, Elisabeth Le Rumeur
Dystrophin and Spectrin are two proteins essential for the organization of the cytoskeleton and for the stabilization of membrane cells. The comparison of these two sister proteins, and with the dystrophin homologue utrophin, enables us to emphasise that, despite a similar topology with common subdomains and a common structural basis of a three-helix coiled-coil, they show a large range of dissimilarities in terms of genetics, cell expression and higher level structural organisation. Interactions with cellular partners, including proteins and membrane phospholipids, also show both strikingly similar and very different behaviours...
2017: Sub-cellular Biochemistry
https://www.readbyqxmd.com/read/28101425/study-of-in-vitro-rbcs-membrane-elasticity-with-aod-scanning-optical-tweezers
#18
Huadong Song, Ying Liu, Bin Zhang, Kangzhen Tian, Panpan Zhu, Hao Lu, Qi Tang
The elasticity of red cell membrane is a critical physiological index for the activity of RBC. Study of the inherent mechanism for RBCs membrane elasticity transformation is attention-getting all along. This paper proposes an optimized measurement method of erythrocytes membrane shear modulus incorporating acousto-optic deflector (AOD) scanning optical tweezers system. By use of this method, both membrane shear moduli and sizes of RBCs with different in vitro times were determined. The experimental results reveal that the RBCs membrane elasticity and size decline with in vitro time extension...
January 1, 2017: Biomedical Optics Express
https://www.readbyqxmd.com/read/28098556/genetic-defects-in-%C3%AE-spectrin-and-tau-sensitize-c-elegans-axons-to-movement-induced-damage-via-torque-tension-coupling
#19
Michael Krieg, Jan Stühmer, Juan G Cueva, Richard Fetter, Kerri Spilker, Daniel Cremers, Kang Shen, Alexander R Dunn, Miriam B Goodman
Our bodies are in constant motion and so are the neurons that invade each tissue. Motion-induced neuron deformation and damage are associated with several neurodegenerative conditions. Here, we investigated the question of how the neuronal cytoskeleton protects axons and dendrites from mechanical stress, exploiting mutations in UNC-70 β-spectrin, PTL-1 tau/MAP2-like and MEC-7 β-tubulin proteins in Caenorhabditis elegans. We found that mechanical stress induces supercoils and plectonemes in the sensory axons of spectrin and tau double mutants...
January 18, 2017: ELife
https://www.readbyqxmd.com/read/28090778/three-novel-spectrin-variants-in-jaundiced-neonates
#20
Robert D Christensen, Archana M Agarwal, Hassan M Yaish, N Scott Reading, Elizabeth A O'Brien, Josef T Prchal
Various mutations in the genes encoding alpha spectrin (SPTA1) or beta spectrin (SPTB) are known to cause erythrocyte membrane disorders, sometimes associated with severe neonatal jaundice and anemia. We used a next-generation sequencing panel to evaluate 3 unrelated neonates who had puzzling cases of nonimmune hemolytic jaundice. In each case, we identified novel mutations in either SPTA1 or SPTB. Correlating erythrocyte morphology, clinical course, and computational analysis, we submit that each of the 3 variants is a probable pathogenic cause of the hereditary hemolytic conditions in these patients...
January 1, 2017: Clinical Pediatrics
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