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https://www.readbyqxmd.com/read/28390949/ncls-and-er-a-stressful-relationship
#1
REVIEW
Davide Marotta, Elisa Tinelli, Sara E Mole
The Neuronal Ceroid Lipofuscinoses (NCLs, Batten disease) are a group of inherited neurodegenerative disorders with variable age of onset, characterised by the lysosomal accumulation of autofluorescent ceroid lipopigments. The endoplasmic reticulum (ER) is a critical organelle for normal cell function. Alteration of ER homeostasis leads to accumulation of misfolded protein in the ER and to activation of the unfolded protein response. ER stress and the UPR have recently been linked to the NCLs. In this review, we will discuss the evidence for UPR activation in the NCLs, and address its connection to disease pathogenesis...
April 5, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28376565/implementation-of-a-hydrotherapy-protocol-to-improve-postpartum-pain-management
#2
Meghann Batten, Eleanor Stevenson, Deb Zimmermann, Christine Isaacs
INTRODUCTION: A growing number of women are seeking alternatives to traditional pharmacologic pain management during birth. While there has been an extensive array of nonpharmacologic options developed for labor, there are limited offerings in the postpartum period. The purpose of this quality improvement project was to implement a hydrotherapy protocol in the early postpartum period to improve pain management for women choosing a nonmedicated birth. PROCESS: The postpartum hydrotherapy protocol was initiated in a certified nurse-midwife (CNM) practice in an urban academic medical center...
March 2017: Journal of Midwifery & Women's Health
https://www.readbyqxmd.com/read/28365442/loss-of-cln3-impacts-protein-secretion-in-the-social-amoeba-dictyostelium
#3
Robert J Huber
Neuronal ceroid lipofuscinosis (NCL), also referred to as Batten disease, is the most common form of childhood neurodegeneration. Mutations in CLN3 cause the most prevalent subtype of the disease, which manifests during early childhood and is currently untreatable. The precise function of the CLN3 protein is still not known, which has inhibited the development of targeted therapies. In the social amoeba Dictyostelium discoideum, loss of the CLN3 homolog, Cln3, reduces adhesion during early development, which delays streaming and aggregation...
March 29, 2017: Cellular Signalling
https://www.readbyqxmd.com/read/28301627/single-staged-tunneled-cheek-interpolation-flap-with-cartilage-batten-graft-for-repair-of-nasal-ala-defect
#4
Tatyana A Petukhova, Jayne S Joo, Daniel B Eisen
<p>Surgical defects located within 5 mm of the nasal alar margin are at risk for alar elevation or collapse of the external nasal valve during wound healing. To reduce the chance of such complications, free cartilage grafts may be used as part of the reconstruction. However, if the defect is large enough so that the free cartilage graft does not fill most of the defect, wound contraction can still lead to alar displacement. In these situations, skin may need to be recruited from either the forehead or cheek in the form of an interpolation flap to cover both the free cartilage graft and the residual cutaneous defect...
March 1, 2017: Journal of Drugs in Dermatology: JDD
https://www.readbyqxmd.com/read/28230600/nasal-valve-collapse-our-treatment-protocol
#5
Giulia Amodeo, Domenico Scopelliti
The nasal valve area is the functional area that allows airflow regulation. It could be divided in an internal area and an external one, basing on the anatomical landmarks. Many conditions can damage these areas with a consequently nasal obstruction, more often in particular extended rhinoplasty and sequels of facial trauma. To restore this area many techniques were advocated during the last decades.In this article the authors investigate on valve areas deficiency in sequel of rhinoplasty proposing a structural approach through alar batten grafts to correct internal-external nasal valves collapse...
February 22, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28213849/widespread-expression-of-a-membrane-tethered-version-of-the-soluble-lysosomal-enzyme-palmitoyl-protein-thioesterase-1
#6
Charles Shyng, Shannon L Macauley, Joshua T Dearborn, Mark S Sands
"Cross-correction," the transfer of soluble lysosomal enzymes between neighboring cells, forms the foundation for therapeutics of lysosomal storage disorders (LSDs). However, "cross-correction" poses a significant barrier to studying the role of specific cell types in LSD pathogenesis. By expressing the native enzyme in only one cell type, neighboring cell types are invariably corrected. In this study, we present a strategy to limit "cross-correction" of palmitoyl-protein thioesterase-1(PPT1), a lysosomal hydrolase deficient in Infantile Neuronal Ceroid Lipofuscinosis (INCL, Infantile Batten disease) to the lysosomal membrane via the C-terminus of lysosomal associated membrane protein-1 (LAMP1)...
February 18, 2017: JIMD Reports
https://www.readbyqxmd.com/read/28165011/mtorc1-independent-tfeb-activation-via-akt-inhibition-promotes-cellular-clearance-in-neurodegenerative-storage-diseases
#7
Michela Palmieri, Rituraj Pal, Hemanth R Nelvagal, Parisa Lotfi, Gary R Stinnett, Michelle L Seymour, Arindam Chaudhury, Lakshya Bajaj, Vitaliy V Bondar, Laura Bremner, Usama Saleem, Dennis Y Tse, Deepthi Sanagasetti, Samuel M Wu, Joel R Neilson, Fred A Pereira, Robia G Pautler, George G Rodney, Jonathan D Cooper, Marco Sardiello
Neurodegenerative diseases characterized by aberrant accumulation of undigested cellular components represent unmet medical conditions for which the identification of actionable targets is urgently needed. Here we identify a pharmacologically actionable pathway that controls cellular clearance via Akt modulation of transcription factor EB (TFEB), a master regulator of lysosomal pathways. We show that Akt phosphorylates TFEB at Ser467 and represses TFEB nuclear translocation independently of mechanistic target of rapamycin complex 1 (mTORC1), a known TFEB inhibitor...
February 6, 2017: Nature Communications
https://www.readbyqxmd.com/read/28134644/management-of-rhinophyma-outcomes-study-of-the-subunit-method
#8
Aladdin H Hassanein, Raj M Vyas, Jessica Erdmann-Sager, Edward J Caterson, Julian J Pribaz
BACKGROUND: Rhinophyma causes a nasal deformity and functional airway obstruction. Partial excision (eg, tangential) with secondary healing commonly removes hypertrophic soft tissues but does not improve nasal support. The subunit method for rhinophyma uses 6 nasal flaps to provide exposure for removal of rhinophymatous tissue and enhance structure. The purpose of this study was to evaluate outcomes of subunit method. METHODS: Medical records of patients with rhinophyma treated with the subunit method between 2013 and 2016 were analyzed...
January 27, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28131750/efficacy-of-eplerenone-in-the-management-of-mineralocorticoid-excess-in-men-with-metastatic-castration-resistant-prostate-cancer-treated-with-abiraterone-without-prednisone
#9
David Gill, David Gaston, Erin Bailey, Andrew Hahn, Sumati Gupta, Julia Batten, Anitha Alex, Kenneth Boucher, David Stenehjem, Neeraj Agarwal
BACKGROUND: Abiraterone acetate has been approved for metastatic castration-resistant prostate cancer (mCRPC). Coadministration with prednisone has been recommended to prevent the toxicity from secondary mineralocorticoid excess, such as hypertension, hypokalemia, and edema. However, the use of prednisone is often not desired by patients because of the potential for detrimental effects of long-term therapy with corticosteroids, especially in those with comorbidities such as diabetes or who have received previous immunotherapeutic agents...
January 5, 2017: Clinical Genitourinary Cancer
https://www.readbyqxmd.com/read/28079639/batten-stitch-a-tissue-sparing-technique-for-correction-of-an-inverted-dog-ear
#10
Michael B Mortazie, Jeremy C Davis, Richard G Bennett
No abstract text is available yet for this article.
January 10, 2017: Dermatologic Surgery: Official Publication for American Society for Dermatologic Surgery [et Al.]
https://www.readbyqxmd.com/read/28065762/characterisation-of-early-changes-in-ovine-cln5-and-cln6-batten-disease-neural-cultures-for-the-rapid-screening-of-therapeutics
#11
Hannah L Best, Nicole J Neverman, Hollie E Wicky, Nadia L Mitchell, Beulah Leitch, Stephanie M Hughes
Batten disease (neuronal ceroid lipofuscinosis) refers to a group of neurodegenerative lysosomal storage diseases predominantly affecting children. There are currently no effective treatments, and the functions of many of the associated gene products are unknown. Here we characterise fetal neural cultures from two genetically distinct sheep forms of Batten disease, with mutations in the lysosomal protein encoding gene CLN5 and endoplasmic reticulum membrane protein encoding gene CLN6, respectively. We found similar reductions in autophagy, acidic organelles and synaptic recycling in both forms compared to unaffected cells...
January 5, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/27913435/idh2-mutations-define-a-unique-subtype-of-breast-cancer-with-altered-nuclear-polarity
#12
Sarah Chiang, Britta Weigelt, Huei-Chi Wen, Fresia Pareja, Ashwini Raghavendra, Luciano G Martelotto, Kathleen A Burke, Thais Basili, Anqi Li, Felipe C Geyer, Salvatore Piscuoglio, Charlotte K Y Ng, Achim A Jungbluth, Jörg Balss, Stefan Pusch, Gabrielle M Baker, Kimberly S Cole, Andreas von Deimling, Julie M Batten, Jonathan D Marotti, Hwei-Choo Soh, Benjamin L McCalip, Jonathan Serrano, Raymond S Lim, Kalliopi P Siziopikou, Song Lu, Xiaolong Liu, Tarek Hammour, Edi Brogi, Matija Snuderl, A John Iafrate, Jorge S Reis-Filho, Stuart J Schnitt
Solid papillary carcinoma with reverse polarity (SPCRP) is a rare breast cancer subtype with an obscure etiology. In this study, we sought to describe its unique histopathologic features and to identify the genetic alterations that underpin SPCRP using massively parallel whole-exome and targeted sequencing. The morphologic and immunohistochemical features of SPCRP support the invasive nature of this subtype. Ten of 13 (77%) SPCRPs harbored hotspot mutations at R172 of the isocitrate dehydrogenase IDH2, of which 8 of 10 displayed concurrent pathogenic mutations affecting PIK3CA or PIK3R1 One of the IDH2 wild-type SPCRPs harbored a TET2 Q548* truncating mutation coupled with a PIK3CA H1047R hotspot mutation...
December 15, 2016: Cancer Research
https://www.readbyqxmd.com/read/27895260/coordination-polymers-and-metal-organic-frameworks-materials-by-design
#13
Stuart R Batten, Neil R Champness
No abstract text is available yet for this article.
January 13, 2017: Philosophical Transactions. Series A, Mathematical, Physical, and Engineering Sciences
https://www.readbyqxmd.com/read/27881166/using-the-social-amoeba-dictyostelium-to-study-the-functions-of-proteins-linked-to-neuronal-ceroid-lipofuscinosis
#14
REVIEW
Robert J Huber
Neuronal ceroid lipofuscinosis (NCL), also known as Batten disease, is a debilitating neurological disorder that affects both children and adults. Thirteen genetically distinct genes have been identified that when mutated, result in abnormal lysosomal function and an excessive accumulation of ceroid lipofuscin in neurons, as well as other cell types outside of the central nervous system. The NCL family of proteins is comprised of lysosomal enzymes (PPT1/CLN1, TPP1/CLN2, CTSD/CLN10, CTSF/CLN13), proteins that peripherally associate with membranes (DNAJC5/CLN4, KCTD7/CLN14), a soluble lysosomal protein (CLN5), a protein present in the secretory pathway (PGRN/CLN11), and several proteins that display different subcellular localizations (CLN3, CLN6, MFSD8/CLN7, CLN8, ATP13A2/CLN12)...
November 24, 2016: Journal of Biomedical Science
https://www.readbyqxmd.com/read/27850212/572-what-does-treatable-mean-a-qualitative-study-of-physician-patient-communication
#15
Katherine Kruse, Jason Batten, Stephanie Kraft, Bela Fishbeyn, David Magnus
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
https://www.readbyqxmd.com/read/27804148/efficacy-of-phosphodiesterase-4-inhibitors-in-juvenile-batten-disease-cln3
#16
Amy Aldrich, Megan E Bosch, Rachel Fallet, Jessica Odvody, Maria Burkovetskaya, Kakulavarapu V Rama Rao, Jonathan D Cooper, Arlene V Drack, Tammy Kielian
OBJECTIVE: Juvenile neuronal ceroid lipofuscinosis (JNCL), or juvenile Batten disease, is a pediatric lysosomal storage disease caused by autosomal recessive mutations in CLN3, typified by blindness, seizures, progressive cognitive and motor decline, and premature death. Currently, there is no treatment for JNCL that slows disease progression, which highlights the need to explore novel strategies to extend the survival and quality of life of afflicted children. Cyclic adenosine monophosphate (cAMP) is a second messenger with pleiotropic effects, including regulating neuroinflammation and neuronal survival...
December 2016: Annals of Neurology
https://www.readbyqxmd.com/read/27766444/flunarizine-rescues-reduced-lifespan-in-cln3-triple-knock-out-caenorhabditis-elegans-model-of-batten-disease
#17
Young Joon Kwon, Marni J Falk, Michael J Bennett
CLN3 disease (Spielmeyer-Vogt-Sjogren-Batten disease, previously known as classic juvenile neuronal ceroid lipofuscinosis, NCL) is a pediatric-onset progressive neurodegenerative disease characterized by progressive vision loss, seizures, loss of cognitive and motor function, and early death. While no precise biochemical mechanism or therapies are known, the pathogenesis of CLN3 disease involves intracellular calcium accumulation that may trigger apoptosis. Our prior work in in vitro cell models of CLN3 deficiency suggested that FDA-approved calcium channel antagonists may have therapeutic value...
March 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/27744565/a-phase-i-study-to-determine-the-pharmacokinetics-and-urinary-excretion-of-belinostat-and-metabolites-in-patients-with-advanced-solid-tumors
#18
Hanna Bailey, Jordan P McPherson, Erin B Bailey, Theresa L Werner, Sumati Gupta, Julia Batten, Guru Reddy, Gajanan Bhat, Sunil Sharma, Neeraj Agarwal
PURPOSE: Belinostat is an inhibitor of histone deacetylase enzymes, resulting in DNA repair inhibition and apoptosis. Present data are lacking to provide dosing recommendations in renal insufficiency. The purpose of this trial was to assess the pharmacokinetics (PK) of belinostat and belinostat metabolites in plasma and urine. METHODS: This was a phase I, single-center, open-label, two-part study. In Part I, patients received single-agent belinostat 1000 mg/m(2)...
October 15, 2016: Cancer Chemotherapy and Pharmacology
https://www.readbyqxmd.com/read/27669405/aberrant-adhesion-impacts-early-development-in-a-dictyostelium-model-for-juvenile-neuronal-ceroid-lipofuscinosis
#19
Robert J Huber, Michael A Myre, Susan L Cotman
Neuronal ceroid lipofuscinosis (NCL), also known as Batten disease, refers to a group of severe neurodegenerative disorders that primarily affect children. The most common subtype of the disease is caused by loss-of-function mutations in CLN3, which is conserved across model species from yeast to human. The precise function of the CLN3 protein is not known, which has made targeted therapy development challenging. In the social amoeba Dictyostelium discoideum, loss of Cln3 causes aberrant mid-to-late stage multicellular development...
September 26, 2016: Cell Adhesion & Migration
https://www.readbyqxmd.com/read/27665766/porous-polyrotaxane-coordination-networks-containing-two-distinct-conformers-of-a-discontinuously-flexible-ligand
#20
Chris S Hawes, Gregory P Knowles, Alan L Chaffee, Keith F White, Brendan F Abrahams, Stuart R Batten, David R Turner
A new divergent homopiperazine-derived ligand N,N'-bis(4-carboxyphenyl)-1,4-diazacycloheptane H2L has been prepared, containing a semirigid saturated heterocyclic core which is capable of providing multiple distinct bridging geometries. Reaction of H2L with zinc acetate in DMSO gives a two-dimensional parallel interpenetrated polyrotaxane structure 1 in which the loops and rods are formed by the bent cis-(eq,ax) twist boat and trans-(ax,ax) twist chair conformers, respectively. By matching the distances between the solvated metal sites in the structure of 1, a related material 2 can be prepared incorporating the pillaring ligand trans-1,2-bis(4-pyridyl)ethylene bpe...
October 17, 2016: Inorganic Chemistry
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