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https://www.readbyqxmd.com/read/29041969/glial-cells-are-functionally-impaired-in-juvenile-neuronal-ceroid-lipofuscinosis-and-detrimental-to-neurons
#1
Lotta Parviainen, Sybille Dihanich, Greg W Anderson, Andrew M Wong, Helen R Brooks, Rosella Abeti, Payam Rezaie, Giovanna Lalli, Simon Pope, Simon J Heales, Hannah M Mitchison, Brenda P Williams, Jonathan D Cooper
The neuronal ceroid lipofuscinoses (NCLs or Batten disease) are a group of inherited, fatal neurodegenerative disorders of childhood. In these disorders, glial (microglial and astrocyte) activation typically occurs early in disease progression and predicts where neuron loss subsequently occurs. We have found that in the most common juvenile form of NCL (CLN3 disease or JNCL) this glial response is less pronounced in both mouse models and human autopsy material, with the morphological transformation of both astrocytes and microglia severely attenuated or delayed...
October 17, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29025946/complete-genome-sequence-of-peste-des-petits-ruminants-virus-from-georgia-2016
#2
Paulina Z Rajko-Nenow, Tabitha G Cunliffe, John T Flannery, Honorata M Ropiak, Lasha Avaliani, Marina Donduashvili, Michael D Baron, Carrie A Batten
We report here the complete genome sequence of a peste des petits ruminants virus (PPRV) from the first outbreak of the disease in Georgia in January 2016. Genome sequencing was performed using Illumina next-generation sequencing technology in conjunction with Sanger sequencing. This PPRV/Georgia/Tbilisi/2016 genome sequence clustered within lineage IV PPRV viruses.
October 12, 2017: Genome Announcements
https://www.readbyqxmd.com/read/28984877/approaches-to-end-of-life-discussions-with-parents-of-a-profoundly-compromised-newborn
#3
J J Paris, V Pai, B M Cummings, J Batten, W E Benitz
No abstract text is available yet for this article.
October 2017: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/28948088/linking-kindling-to-increased-glutamate-release-in-the-dentate-gyrus-of-the-hippocampus-through-the-stxbp5-tomosyn-1-gene
#4
Seth R Batten, Elena A Matveeva, Sidney W Whiteheart, Thomas C Vanaman, Greg A Gerhardt, John T Slevin
INTRODUCTION: In kindling, repeated electrical stimulation of certain brain areas causes progressive and permanent intensification of epileptiform activity resulting in generalized seizures. We focused on the role(s) of glutamate and a negative regulator of glutamate release, STXBP5/tomosyn-1, in kindling. METHODS: Stimulating electrodes were implanted in the amygdala and progression to two successive Racine stage 5 seizures was measured in wild-type and STXBP5/tomosyn-1(-/-) (Tom(-/-)) animals...
September 2017: Brain and Behavior
https://www.readbyqxmd.com/read/28930889/the-burden-of-mental-illness-among-veterans-use-of-vha-health-care-services-by-those-with-service-connected-conditions
#5
Charles Maynard, Adam Batten, Chuan-Fen Liu, Karin Nelson, Stephan D Fihn
BACKGROUND: Little is known about how Veterans with service-connected conditions use health care provided by the Veterans Health Administration (VHA). OBJECTIVES: To ascertain what proportion of Veterans with service-connected conditions used VHA health care and whether it varied according to type of condition, combined disability rating, age, sex, military rank, or other characteristics and whether there were differences in receipt of inpatient and outpatient care...
November 2017: Medical Care
https://www.readbyqxmd.com/read/28913343/exacerbating-and-reversing-lysosomal-storage-diseases-from-yeast-to-humans
#6
REVIEW
Tamayanthi Rajakumar, Andrew B Munkacsi, Stephen L Sturley
Lysosomal storage diseases (LSDs) arise from monogenic deficiencies in lysosomal proteins and pathways and are characterized by a tissue-wide accumulation of a vast variety of macromolecules, normally specific to each genetic lesion. Strategies for treatment of LSDs commonly depend on reduction of the offending metabolite(s) by substrate depletion or enzyme replacement. However, at least 44 of the ~50 LSDs are currently recalcitrant to intervention. Murine models have provided significant insights into our understanding of many LSD mechanisms; however, these systems do not readily permit phenotypic screening of compound libraries, or the establishment of genetic or gene-environment interaction networks...
August 25, 2017: Microbial Cell
https://www.readbyqxmd.com/read/28828392/thermal-stability-as-a-determinant-of-aav-serotype-identity
#7
Antonette Bennett, Saajan Patel, Mario Mietzsch, Ariana Jose, Bridget Lins-Austin, Jennifer C Yu, Brian Bothner, Robert McKenna, Mavis Agbandje-McKenna
Currently, there are over 150 ongoing clinical trials utilizing adeno-associated viruses (AAVs) to target various genetic diseases, including hemophilia (AAV2 and AAV8), congenital heart failure (AAV1 and AAV6), cystic fibrosis (AAV2), rheumatoid arthritis (AAV2), and Batten disease (AAVrh.10). Prior to patient administration, AAV vectors must have their serotype, concentration, purity, and stability confirmed. Here, we report the application of differential scanning fluorimetry (DSF) as a good manufacturing practice (GMP) capable of determining the melting temperature (Tm) for AAV serotype identification...
September 15, 2017: Molecular Therapy. Methods & Clinical Development
https://www.readbyqxmd.com/read/28812237/pharmacological-effects-on-ceroid-lipofuscin-and-neuronal-structure-in-cln3-%C3%A2-ex7-8-mouse-brain-cultures
#8
Douglas E Brenneman, David A Pearce, Attila Kovacs, Shawn DeFrees
Juvenile Batten disease (JBD) is an inherited disorder that is characterized by the development of blindness, seizures, and progressive motor, psychiatric, and cognitive impairment. A model of JBD expressing the predominant human mutation (Cln3 (∆ex7/8) ) has been explored. Dissociated brain cultures from Cln3 (∆ex7/8) knock-in mice were compared to wild type (WT) for effects on granules of ceroid lipofuscin (CL) and neuronal structure. Utilizing high content image analysis of CL granules identified with antibodies to mitochondrial ATP synthase subunit c or tripeptidyl peptidase-1, significant increases in the areas for both immunoreactive granules were observed in Cln3 (∆ex7/8) cultures in comparison to WT...
August 15, 2017: Journal of Molecular Neuroscience: MN
https://www.readbyqxmd.com/read/28791757/differential-stimulus-control-of-drug-seeking-multimodal-reinstatement
#9
Seth R Batten, Joshua S Beckmann
In animal models of substance-use disorder, individuals that repeatedly self-administer drugs of abuse have long-lasting neuronal adaptations that do not occur ostensibly in control animals only exposed to natural reinforcers (e.g. food). Because any treatment for substance-use disorder will be given to individuals with drug-taking histories, adequate dissociation of the specific neurobehavioral mechanisms underlying drug reinforcement, natural reinforcement and their associated cue effects requires an experimental model that exposes individuals to both reinforcer conditions, along with their associated stimuli...
August 9, 2017: Addiction Biology
https://www.readbyqxmd.com/read/28737555/l-shaped-septal-extension-spreader-graft-for-improvement-of-tip-symmetry-in-unilateral-cleft-lip-nose-deformities
#10
Serkan Sertel, Ioana-Irina Venara-Vulpe, François Gorostidi, Anthony de Buys Roessingh, Philippe Pasche
INTRODUCTION: The unilateral cleft lip nose is characterized by numerous complex and interdependent deformities. Secondary rhinoplasty techniques aim to correct cleft lip nose deformities by using multiple maneuvers combining septum and nasal spine medialization and alar cartilage, as well as soft tissue mobilization and repositioning. Moreover, cartilage grafting is frequently used to restore adequate tip projection and nasal symmetry. We present a technique of cartilage grafting commonly used in noncleft rhinoplasties that we modified for cases of moderate cleft lip nose deformities...
July 22, 2017: Annals of Plastic Surgery
https://www.readbyqxmd.com/read/28677990/elements-of-the-veterans-health-administration-patient-centered-medical-home-are-associated-with-greater-adherence-to-oral-hypoglycemic-agents-in-patients-with-diabetes
#11
Nicholas Meo, Edwin Wong, Haili Sun, Idamay Curtis, Adam Batten, Stephan D Fihn, Karin Nelson
In 2010, Veterans Health Administration (VHA) primary care clinics adopted a patient-centered medical home (PCMH) model. This study sought to examine the association between the organizational features related to adoption of PCMH and the level of adherence to oral hypoglycemic agents (OHAs) among patients with diabetes. This retrospective cohort study involved 757 VA clinics that provide primary care to 440,971 patients with diabetes who were taking OHAs in fiscal year 2012. One-year refill-based medication possession ratios (MPRs) were calculated at the patient level...
July 5, 2017: Population Health Management
https://www.readbyqxmd.com/read/28667019/retrotransposons-are-the-major-contributors-to-the-expansion-of-the-drosophila-ananassae-muller-f-element
#12
Wilson Leung, Christopher D Shaffer, Elizabeth J Chen, Thomas J Quisenberry, Kevin Ko, John M Braverman, Thomas C Giarla, Nathan T Mortimer, Laura K Reed, Sheryl T Smith, Srebrenka Robic, Shannon R McCartha, Danielle R Perry, Lindsay M Prescod, Zenyth A Sheppard, Ken J Saville, Allison McClish, Emily A Morlock, Victoria R Sochor, Brittney Stanton, Isaac C Veysey-White, Dennis Revie, Luis A Jimenez, Jennifer J Palomino, Melissa D Patao, Shane M Patao, Edward T Himelblau, Jaclyn D Campbell, Alexandra L Hertz, Maddison F McEvilly, Allison R Wagner, James Youngblom, Baljit Bedi, Jeffery Bettincourt, Erin Duso, Maiye Her, William Hilton, Samantha House, Masud Karimi, Kevin Kumimoto, Rebekah Lee, Darryl Lopez, George Odisho, Ricky Prasad, Holly Lyn Robbins, Tanveer Sandhu, Tracy Selfridge, Kara Tsukashima, Hani Yosif, Nighat P Kokan, Latia Britt, Alycia Zoellner, Eric P Spana, Ben T Chlebina, Insun Chong, Harrison Friedman, Danny A Mammo, Chun L Ng, Vinayak S Nikam, Nicholas U Schwartz, Thomas Q Xu, Martin G Burg, Spencer M Batten, Lindsay M Corbeill, Erica Enoch, Jesse J Ensign, Mary E Franks, Breanna Haiker, Judith A Ingles, Lyndsay D Kirkland, Joshua M Lorenz-Guertin, Jordan Matthews, Cody M Mittig, Nicholaus Monsma, Katherine J Olson, Guillermo Perez-Aragon, Alen Ramic, Jordan R Ramirez, Christopher Scheiber, Patrick A Schneider, Devon E Schultz, Matthew Simon, Eric Spencer, Adam C Wernette, Maxine E Wykle, Elizabeth Zavala-Arellano, Mitchell J McDonald, Kristine Ostby, Peter Wendland, Justin R DiAngelo, Alexis M Ceasrine, Amanda H Cox, James E B Docherty, Robert M Gingras, Stephanie M Grieb, Michael J Pavia, Casey L Personius, Grzegorz L Polak, Dale L Beach, Heaven L Cerritos, Edward A Horansky, Karim A Sharif, Ryan Moran, Susan Parrish, Kirsten Bickford, Jennifer Bland, Juliana Broussard, Kerry Campbell, Katelynn E Deibel, Richard Forka, Monika C Lemke, Marlee B Nelson, Catherine O'Keeffe, S Mariel Ramey, Luke Schmidt, Paola Villegas, Christopher J Jones, Stephanie L Christ, Sami Mamari, Adam S Rinaldi, Ghazal Stity, Amy T Hark, Mark Scheuerman, S Catherine Silver Key, Briana D McRae, Adam S Haberman, Sam Asinof, Harriette Carrington, Kelly Drumm, Terrance Embry, Richard McGuire, Drew Miller-Foreman, Stella Rosen, Nadia Safa, Darrin Schultz, Matt Segal, Yakov Shevin, Petros Svoronos, Tam Vuong, Gary Skuse, Don W Paetkau, Rachael K Bridgman, Charlotte M Brown, Alicia R Carroll, Francesca M Gifford, Julie Beth Gillespie, Susan E Herman, Krystal L Holtcamp, Misha A Host, Gabrielle Hussey, Danielle M Kramer, Joan Q Lawrence, Madeline M Martin, Ellen N Niemiec, Ashleigh P O'Reilly, Olivia A Pahl, Guadalupe Quintana, Elizabeth A S Rettie, Torie L Richardson, Arianne E Rodriguez, Mona O Rodriguez, Laura Schiraldi, Joanna J Smith, Kelsey F Sugrue, Lindsey J Suriano, Kaitlyn E Takach, Arielle M Vasquez, Ximena Velez, Elizabeth J Villafuerte, Laura T Vives, Victoria R Zellmer, Jeanette Hauke, Charles R Hauser, Karolyn Barker, Laurie Cannon, Perouza Parsamian, Samantha Parsons, Zachariah Wichman, Christopher W Bazinet, Diana E Johnson, Abubakarr Bangura, Jordan A Black, Victoria Chevee, Sarah A Einsteen, Sarah K Hilton, Max Kollmer, Rahul Nadendla, Joyce Stamm, Antoinette E Fafara-Thompson, Amber M Gygi, Emmy E Ogawa, Matt Van Camp, Zuzana Kocsisova, Judith L Leatherman, Cassie M Modahl, Michael R Rubin, Susana S Apiz-Saab, Suzette M Arias-Mejias, Carlos F Carrion-Ortiz, Patricia N Claudio-Vazquez, Debbie M Espada-Green, Marium Feliciano-Camacho, Karina M Gonzalez-Bonilla, Mariela Taboas-Arroyo, Dorianmarie Vargas-Franco, Raquel Montañez-Gonzalez, Joseph Perez-Otero, Myrielis Rivera-Burgos, Francisco J Rivera-Rosario, Heather L Eisler, Jackie Alexander, Samatha K Begley, Deana Gabbard, Robert J Allen, Wint Yan Aung, William D Barshop, Amanda Boozalis, Vanessa P Chu, Jeremy S Davis, Ryan N Duggal, Robert Franklin, Katherine Gavinski, Heran Gebreyesus, Henry Z Gong, Rachel A Greenstein, Averill D Guo, Casey Hanson, Kaitlin E Homa, Simon C Hsu, Yi Huang, Lucy Huo, Sarah Jacobs, Sasha Jia, Kyle L Jung, Sarah Wai-Chee Kong, Matthew R Kroll, Brandon M Lee, Paul F Lee, Kevin M Levine, Amy S Li, Chengyu Liu, Max Mian Liu, Adam P Lousararian, Peter B Lowery, Allyson P Mallya, Joseph E Marcus, Patrick C Ng, Hien P Nguyen, Ruchik Patel, Hashini Precht, Suchita Rastogi, Jonathan M Sarezky, Adam Schefkind, Michael B Schultz, Delia Shen, Tara Skorupa, Nicholas C Spies, Gabriel Stancu, Hiu Man Vivian Tsang, Alice L Turski, Rohit Venkat, Leah E Waldman, Kaidi Wang, Tracy Wang, Jeffrey W Wei, Dennis Y Wu, David D Xiong, Jack Yu, Karen Zhou, Gerard P McNeil, Robert W Fernandez, Patrick Gomez Menzies, Tingting Gu, Jeremy Buhler, Elaine R Mardis, Sarah C R Elgin
The discordance between genome size and the complexity of eukaryotes can partly be attributed to differences in repeat density. The Muller F element (∼5.2 Mb) is the smallest chromosome in Drosophila melanogaster, but it is substantially larger (>18.7 Mb) in D. ananassae To identify the major contributors to the expansion of the F element and to assess their impact, we improved the genome sequence and annotated the genes in a 1.4-Mb region of the D. ananassae F element, and a 1.7-Mb region from the D element for comparison...
August 7, 2017: G3: Genes—Genomes—Genetics
https://www.readbyqxmd.com/read/28660435/non-operative-management-of-distal-humerus-fractures-in-the-elderly-a-review-of-functional-outcomes
#13
Timothy J Batten, Claire Sin-Hidge, Mark D Brinsden, Paul M Guyver
Comminuted distal humerus fractures in the elderly have traditionally been managed by ORIF or total elbow arthroplasty (TEA). This poses a treatment dilemma in elderly patients where anaesthetic and surgical risks combine with poor bone and wound healing. We aimed to assess the functional outcomes in patients managed non-operatively, with TEA being used as the salvage procedure. Retrospective analysis of patients over 65 years presenting to our unit between 2005 and 2015 was undertaken. Sixty-two patients were identified, 38 had died, and 5 were lost to follow-up leaving 5 with immediate TEA and 14 non-operatively managed, available for review...
June 28, 2017: European Journal of Orthopaedic Surgery & Traumatology: Orthopédie Traumatologie
https://www.readbyqxmd.com/read/28623936/safety-and-potential-efficacy-of-gemfibrozil-as-a-supportive-treatment-for-children-with-late-infantile-neuronal-ceroid-lipofuscinosis-and-other-lipid-storage-disorders
#14
REVIEW
Kyeongsoon Kim, Hynda K Kleinman, Hahn-Jun Lee, Kalipada Pahan
Neuronal Ceroid Lipofuscinosis (NCL), also known as Batten disease, is a group of genetically distinct lysosomal disorders that mainly affect the central nervous system, resulting in progressive motor and cognitive decline primarily in children. Multiple distinct genes involved in the metabolism of lipids have been identified to date with various mutations in this family of diseases. There is no cure for these diseases but some new therapeutic approaches have been tested that offer more hope than the standard palliative care...
June 17, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28559377/calcineurin-nfat-signaling-in-activated-astrocytes-drives-network-hyperexcitability-in-a%C3%AE-bearing-mice
#15
Pradoldej Sompol, Jennifer L Furman, Melanie M Pleiss, Susan D Kraner, Irina A Artiushin, Seth R Batten, Jorge E Quintero, Linda A Simmerman, Tina L Beckett, Mark A Lovell, M Paul Murphy, Greg A Gerhardt, Christopher M Norris
Hyperexcitable neuronal networks are mechanistically linked to the pathologic and clinical features of Alzheimer's disease (AD). Astrocytes are a primary defense against hyperexcitability, but their functional phenotype during AD is poorly understood. Here, we found that activated astrocytes in the 5xFAD mouse model were strongly associated with proteolysis of the protein phosphatase calcineurin (CN) and the elevated expression of the CN-dependent transcription factor nuclear factor of activated T cells 4 (NFAT4)...
June 21, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28520827/surgical-outcomes-of-bony-batten-grafting-to-correct-caudal-septal-deviation-in-septoplasty
#16
Do-Youn Kim, Sung Hoon Nam, Sami E Alharethy, Yong Ju Jang
Improtance: Correction of caudal septal deviation with a batten graft has been popularized recently. However, few reports have documented the surgical outcomes of this technique, especially the use of bony batten grafts in septoplasty. Objective: To evaluate the surgical outcomes of bony batten grafting for the management of caudal septal deviation in endonasal septoplasty. Design, Setting, and Participants: This retrospective cohort study evaluates the medical records of 141 patients with caudal septal deviation who underwent septoplasty using bony batten grafts from September 1, 2011, through February 29, 2016, at a tertiary referral hospital...
May 18, 2017: JAMA Facial Plastic Surgery
https://www.readbyqxmd.com/read/28476898/response-to-don-t-let-kids-play-football-a-killer-idea
#17
EDITORIAL
Rachael Bullingham, Adam White, John Batten
No abstract text is available yet for this article.
October 2017: British Journal of Sports Medicine
https://www.readbyqxmd.com/read/28468207/management-of-rhinophyma-outcomes-study-of-the-subunit-method
#18
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...
May 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28464005/a-tailored-mouse-model-of-cln2-disease-a-nonsense-mutant-for-testing-personalized-therapies
#19
Ryan D Geraets, Logan M Langin, Jacob T Cain, Camille M Parker, Rosanna Beraldi, Attila D Kovacs, Jill M Weimer, David A Pearce
The Neuronal Ceroid Lipofuscinoses (NCLs), also known as Batten disease, result from mutations in over a dozen genes. Although, adults are susceptible, the NCLs are frequently classified as pediatric neurodegenerative diseases due to their greater pediatric prevalence. Initial clinical presentation usually consists of either seizures or retinopathy but develops to encompass both in conjunction with declining motor and cognitive function. The NCLs result in premature death due to the absence of curative therapies...
2017: PloS One
https://www.readbyqxmd.com/read/28456990/prenatal-diagnosis-of-lysosomal-storage-disorders-using-chorionic-villi
#20
Jyotsna Verma, Sunita Bijarnia-Mahay, Ishwar C Verma
Prenatal enzymatic diagnosis for an array of lysosomal storage disorders (LSDs) can be performed accurately, provided that a confirmed diagnosis by biochemical/molecular study in the index case is available and a strict defined protocol, specific to each individual disorder is followed. The present chapter describes the protocols for reliable and accurate prenatal enzymatic diagnoses by fluorometric and spectrophotometric methods of lysosomal storage disorders: Gaucher, Fabry, Pompe, Niemann Pick A/B, Tay Sach, Sandhoff, GM1, Mucoplysaccharidoses, Wolman, Krabbe, Metachromatic leukodystrophy, and Batten diseases using uncultured chorionic villi samples...
2017: Methods in Molecular Biology
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