tbuckley/cabot-search huggingface dataset:
52. AAV-Delivered RNAi Improves Cellular and Motor Phenotypes in a Mouse Model for Huntington's Disease
Authors: Scott Q. Harper, Patrick D. Staber, Xiaohua He, Ines H. Martins, Qinwen Mao, Henry L. Paulson, Robert M. Kotin, Beverly L. Davidson
Journal: Molecular Therapy (2005)

Top of pageAbstract
Huntington's Disease (HD) is a fatal, dominant neurodegenerative disorder caused by polyglutamine expansion (CAG codon, Q) in the protein huntingtin (htt). Hallmark HD characteristics include cognitive and behavioral disturbance, involuntary movements (chorea), neuronal inclusions, and striatal and cortical neurodegeneration. Therapies affecting events downstream of disease allele expression have been tested in HD animal models, but no effective treatment currently exists. Moreover, no therapy has been described that directly reduces mutant huntingtin gene expression, thereby targeting the fundamental, underlying pathological insult. RNA interference (RNAi) has emerged as a potential therapeutic tool for treating dominant diseases by directly reducing disease gene expression. We hypothesized that reduction of mutant htt by small hairpin RNA (shRNA) would improve HD-associated phenotypes in a transgenic mouse model for HD (HD-N171-82Q).

We identified a U6 promoter-driven shRNA (shHD2.1) that caused a dose-dependent reduction of human htt transcript and protein in HEK 293 cells in contrast to a control hairpin targeting E. coli |[beta]|-galactosidase. AAV1s expressing shHD2.1 injected into HD-N171-82Q mice reduced human htt transgene levels by |[sim]|50% compared to those injected with AAV1.shLacZ. Additionally, the striatal and cerebellar inclusions evident in untreated or AAVshLacZ treated HD mice were significantly reduced following AAV1.shHD2.1 gene transfer. Finally, AAV1.shHD2.1, but not AAV1.shLacZ, led to a statistically significant correction of HD-associated motor abnormalities in the HD-N171-82Q mice as determined by rotarod performance and gait analysis.

These exciting in vivo results show that shHD2.1 reduces htt mRNA, prevents protein aggregate formation, and improves motor and gait abnormalities in an HD mouse model. Our data provide support for the further development of RNAi for HD therapy.
Mucosal Healing and Risk for Lymphoproliferative Malignancy in Celiac Disease
Authors: Benjamin Lebwohl, Fredrik Granath, Anders Ekbom, Karin E. Smedby, Joseph A. Murray, Alfred I. Neugut, Peter H.R. Green, Jonas F. Ludvigsson
Journal: Annals of Internal Medicine (2013)

Background: Celiac disease (CD) is associated with an increased risk for lymphoproliferative malignancy (LPM). Whether this risk is affected by the results of follow-up intestinal biopsy, performed to document mucosal healing, is unknown. Objective: To examine the association between mucosal healing in CD and subsequent LPM. Design: Population-based cohort study. Setting: 28 pathology departments in Sweden. Patients: 7625 patients with CD who had follow-up biopsy after initial diagnosis. Measurements: The risk for LPM was compared with that of the general population by using expected rates. The rate of LPM in patients with persistent villous atrophy was compared with that of those with mucosal healing by using Cox regression. Results: Among 7625 patients with CD and follow-up biopsy, 3308 (43%) had persistent villous atrophy. The overall risk for LPM was higher than that in the general population (standardized incidence ratio [SIR], 2.81 [95% CI, 2.10 to 3.67]) and was greater among patients with persistent villous atrophy (SIR, 3.78 [CI, 2.71 to 5.12]) than among those with mucosal healing (SIR, 1.50 [CI, 0.77 to 2.62]). Persistent villous atrophy compared with mucosal healing was associated with an increased risk for LPM (hazard ratio [HR], 2.26 [CI, 1.18 to 4.34]). The risk for T-cell lymphoma was increased (HR, 3.51 [CI, 0.75 to 16.34]) but not for B-cell lymphoma (HR, 0.97 [CI, 0.21 to 4.49]). Limitation: No data on dietary adherence. Conclusion: Increased risk for LPM in CD is associated with the follow-up biopsy results, with a higher risk among patients with persistent villous atrophy. Follow-up biopsy may effectively stratify patients with CD by risk for subsequent LPM. Primary Funding Source: National Institutes of Health, The American-Scandinavian Foundation, Celiac Sprue Association, Örebro University Hospital, Karolinska Institutet, Swedish Society of Medicine, Swedish Research Council, and Swedish Celiac Society. Chinese translation
Regulation of lipolysis during the neonatal period. Importance of thyrotropin.
Authors: C Marcus, H Ehrén, P Bolme, P Arner
Journal: Journal of Clinical Investigation (1988)

We investigated the lipolytic effect of several hormones on isolated human adipocytes obtained at different donor ages. In neonates, noradrenaline and adrenaline had an insignificant lipolytic effect (70% over basal). In this age group only thyrotropin (TSH) had a significant effect in physiological concentrations, and the maximal lipolytic effect (700% over basal) was the same as that of isoprenaline. The lipolytic effect of TSH was the same in premature 4-10-wk-old infants with a gestational age of 27-33 wk as in neonates, but fat cells from infants 4-10 wk old, born at term, showed a significantly lower effect. In children and adults, the lipolytic effect of TSH gradually decreased further and was present only in unphysiological concentrations. The catecholamine-induced lipolysis was pronounced and was similar in children and adults (350% over basal). TSH is the dominating lipolytic hormone in vitro during the neonatal period. Thus, the peak elevation of circulating TSH, which is seen immediately after birth, may be essential to lipolysis during this part of life.
Intrapartum Fetoscopy: Technique and Indications
Authors: B. M. Petrikovsky
Journal: Endoscopy (1988)

The present article summarizes six years of experience with intrapartum visualization of the uterus and its contents (fetus, umbilical cord, placenta and amniotic fluid). The choledochofiberscope, previously sterilized in 96% alcohol was introduced into the amniotic cavity via the cervical canal after spontaneous or artificial rupture of the membranes. The following abnormalities were diagnosed with high accuracy: umbilical cord complications (28 cases), placental pathology (36 cases), dehiscence of the uterine scar (2 cases), fetal dysmaturity (19 cases), congenital anomalies (15 cases). No fetal or maternal complications of fetoscopy were noted. Intrapartum fetoscopy can provide the obstetrician with important information in selected cases. Potential uses of intrapartum fetoscopy are discussed.
16S rDNA-Based Phylogeny of Non-Symbiotic Bacteria of Entomopathogenic Nematodes from Infected Insect Cadavers
Authors: M. Razia, R. Karthik Raja, K. Padmanaban, P. Chellapandi, S. Sivaramakrishnan
Journal: Genomics Proteomics & Bioinformatics (2011)

Abstract Using 16S rDNA gene sequencing technique, three different species of non-symbiotic bacteria of entomopathogenic nematodes (EPNs) (Steinernema sp. and Heterorhabditis sp.) were isolated and identified from infected insect cadavers (Galleria mellonella larvae) after 48-hour post infections. Sequence similarity analysis revealed that the strains SRK3, SRK4 and SRK5 belong to Ochrobactrum cytisi, Schineria larvae and Ochrobactrum anthropi, respectively. The isolates O. anthropi and S. larvae were found to be associated with Heterorhabditis indica strains BDU-17 and Yer-136, respectively, whereas O. cytisi was associated with Steinernema siamkayai strain BDU-87. Phenotypically, temporal EPN bacteria were fairly related to symbiotic EPN bacteria (Photorhabdus and Xenorhabdus genera). The strains SRK3 and SRK5 were phylogeographically similar to several non-symbionts and contaminated EPN bacteria isolated in Germany (LMG3311T) and China (X-14), while the strain SRK4 was identical to the isolates of S. larvae (L1/57, L1/58, L1/68 and L2/11) from Wohlfahrtia magnifica in Hungary. The result was further confirmed by RNA secondary structure and minimum energy calculations of aligned sequences. This study suggested that the non-symbionts of these nematodes are phylogeographically diverged in some extent due to phase variation. Therefore, these strains are not host-dependent, but environment-specific isolates.
Kawasaki Syndrome: Description of Two Outbreaks in the United States
Authors: David M. Bell, Edward W. Brink, Joel L. Nitzkin, Caroline Breese Hall, Herta Wulff, Ivor D. Berkowitz, Paul M. Feorino, Robert C. Holman, Cynthia L. Huntley, Richard H. Meade, Larry J. Anderson, Sarah H. Cheeseman, Nicholas J. Fiumara, Robert F. Gilfillan, Daniel E. Keim, John F. Modlin
Journal: New England Journal of Medicine (1981)

Investigation of two outbreaks of Kawasaki syndrome (KS) in the United States in 1979 and in 1980 revealed no evidence of person-to-person transmission or of a common-source exposure among patients. Questionnaire data showed that KS was more likely to occur in children of middle and upper socioeconomic status than in those of lower status (P less than 0.05 and P less than 0.001 for the respective outbreaks) and that patients with KS had a higher incidence of an antecedent, primarily respiratory illness than did controls matched for age, sex, and race (83% of patients in the first outbreak vs. 30% of one control group, P less than 0.01, and vs. 36% of another control group, P less than 0.02; and 56% of patients in the second outbreak vs. 32% of their controls, P less than 0.02). However, laboratory studies did not identify an etiologic agent for either KS or for the antecedent illness that may be a risk factor for KS.
A conversation with Donald Seldin
Authors: Ushma S. Neill
Journal: Journal of Clinical Investigation (2012)

Today we are joined by Donald Seldin from The University of Texas Southwestern Medical School (Figure ​(Figure1).1). Seldin served as the chairman of medicine at UT Southwestern for nearly four decades and transformed the department and the school from a grouping of ramshackle army barracks to a world-class medical center, host to Nobel Laureates and members of the national academies.
Dermatoglyphics in dermatitis herpetiformis
Authors: D.F. ROBERTS, N. ABDULLAH, J. MARKS, S. SHUSTER
Journal: British Journal of Dermatology (1978)

In a dermatoglyphic study of 101 patients with dermatitis herpetiformis, comparison was made with their normal relatives and several random normal series. The results of the several comparisons are quite consistent, considering the nature of the data, for the all point to an attenuation of qualitative and quantitative digital and palmar traits in the patients. It is argued that these differences are associated with the disease itself, and that some of the female relatives have an inherited tendency to the disorder, but do not express it. The findings suggest the involvement of genetic factors in the aetiology, and possibly intrauterine environmental influences as well.
Thymidine phosphorylase expression is associated with time to progression in patients receiving low-dose, docetaxel-modulated capecitabine for metastatic breast cancer
Authors: F. Puglisi, G.G. Cardellino, D. Crivellari, C. Di Loreto, M.D. Magri, A.M. Minisini, M. Mansutti, C. Andreetta, S. Russo, D. Lombardi, T. Perin, G. Damante, A. Veronesi
Journal: Annals of Oncology (2008)
SUCCESFUL TRANSCATHETER THERAPY IN PARTIAL ABNORMAL PULMONARY VENOUS RETURN WITH ADDITIONAL SUPPLY TO LEFT ATRIUM
Authors: Alain Fraisse, Debora Luciano, Daniela Laux, Jean-Rene Lusson, Sebastien Hascoet, Pascal Amedro, Caroline Ovaert, Younes Boudjemline
Journal: Journal of the American College of Cardiology (2013)
MENTAL ILLNESS AND VISUAL ILLUSION
Authors: CHARLES KORENYI
Journal: American Journal of Psychiatry (1963)

The Ames rotating trapezoidal window was demonstrated to 110 acute schizophrenics, 251 chronic psychotics, and 25 controls. The ratio of subjects experiencing the visual illusion was found similar in each group : 97%, 90%, and 96% respectively. It is concluded that mental disorder does not alter this form of illusory perception.
The GMC's stance on Avastin
Authors: N. Dickson
Journal: BMJ (2015)

The European Pharmacopeia surface test to analyze the hydrolytic resistance is a common industrial method to understand and ensure the quality of produced glass vials. Hydrolytic resistance is evaluated by calculating the alkalinity of water extract from autoclaved vials by titration. As an alternative to this titration technique, a conductivity technique was assessed, which directly measures the ions in the water extract. A conductivity meter with a 12 mm diameter electrode was calibrated with a 100 μS/cm conductivity standard and carryover minimized by rinsing the probe in a water beaker per analysis. The limit of quantification at 1 μS/cm was determined as having a signal-to-noise ratio of 3 compared with the water blank. The conductivity method was selective for glass-composing elements (boron, sodium, aluminum, silicon, potassium, and calcium) within the vial extract. Accuracies of spiked conductivity standard within the range of 1 to 100 μS/cm were ±7% and had linearity with coefficient of determination (R<sup>2</sup>) of ≥0.9999. Intraday precision had a relative standard deviation (RSD) (n = 5) of ≤6% for spiked conductivity standard within the range of 1 to 100 μS/cm. Interday precision had a RSD (n = 4) of ≤6% for 10 vials from three glass vial lots. Conductivity of water extracts from nine sets of seven lots of glass vials had a precise linear relationship [R<sup>2</sup> = 0.9876, RSD = 1% (n = 9)] with titration volumes of the same lots. Conductivity results in μS/cm could be converted to titration volumes in milliliters by a conversion factor of 0.0275. The simplicity, sample stability, and individual vial analysis of the conductivity technique were more advantageous than the current titration technique. <i>LAY ABSTRACT:</i> The quality of glass vials used as primary containers in the pharmaceutical industry is of concern due to recent observations of glass flake-like delamination, or <i>lamellae,</i> under specific storage conditions. The current European Pharmacopoeia method to assess glass vial quality utilizes acid titration of vial extract pools to determine hydrolytic resistance or alkalinity. As an alternative to the European Pharmacopoeia method, the vial extracts were analyzed for conductivity, which directly determines the level of ions that were readily extracted from the vial surfaces. Lower quality glass would have greater surface defects that lead to higher ions extracted and higher conductivity value. The conductivity method was found to be suitable to measure the ions in water extracts and showed strong correlation with alkalinity. The advantage of the conductivity method over the alkalinity method was greater ease, lower volume requirements, stability, and flexibility in analysis.
Persistent viremia after recovery from self-limited acute hepatitis B
Authors: Hiroshi Yotsuyanagi, Kiyomi Yasuda, Shiro Iino, Kyoji Moriya, Yoshizumi Shintani, Hajime Fujie, Takeya Tsutsumi, Satoshi Kimura, Kazuhiko Koike
Journal: Hepatology (1998)

To define the duration of viremia in the course of acute hepatitis B, we semiquantitatively determined the levels of hepatitis B virus (HBV) DNA in the sera, using polymerase chain reaction (PCR) coupled with Southern blotting, of non-immunocompromised patients with self-limited acute hepatitis B. In the sera of 10 of 11 patients, HBV DNA, which was presumably coated with viral proteins, was detected for a long period after recovery, even at the final observation times, which ranged from 6 to 19 months after disease onset. To characterize the mode of HBV that was present in serum, we immunoprecipitated immune complexes in sera by the addition of anti-human immunoglobulin G (IgG) and determined the levels of HBV DNA separately in the supernatants and pellets. In the acute phase of hepatitis B, high levels of HBV DNA were detected both in the supernatants and pellets at comparative levels. After the convalescent phase, the amount of HBV DNA in the supernatant decreased with respect to that in the pellets. It is notable that, in most cases, serum HBV persisted as a form of immune complex even after the seroconversion to antibody to hepatitis B surface antigen (anti-HBs). These data suggest that the replication of HBV may persist in some organs, most likely in the liver or peripheral blood cells, for a long period after recovery from acute hepatitis B, and the data indicate the possible transmission of HBV from organ transplantation donors who exhibit serological markers of past infection only.
Prognostic value of normal Tc-99m myoview SPECT imaging: preliminary results from the myoview multicenter registry in 4,728 patients
Authors: Leslee J. Shaw, Robert Hendel, Salvador Borges-Neto, Joy Burnette, Rory Hachamovitch, Elizabeth Krawczynska, Naomi Alazraki, Manuel Cerqueira, Jamshid Maddahi
Journal: Journal of the American College of Cardiology (2002)
Vitamins C and E attenuate plasminogen activator inhibitor-1 (PAI-1) expression in a hypercholesterolemic porcine model of angioplasty
Authors: J Orbe
Journal: Cardiovascular Research (2001)

The plasminogen activator inhibitor-1 (PAI-1), which modulates fibrinolysis and cell migration, may influence proteolysis and neointimal formation in the arterial wall contributing to restenosis after vascular injury. Antioxidants have been proposed as inhibiting multiple proatherogenic events. We explore the effect of vitamins C and E on PAI-1 expression in an experimental model of angioplasty in hypercholesterolemic pigs.A total of 44 Yucatan minipigs were divided into three diet groups: a normal-cholesterol (NC), a high-cholesterol (HC), and a high-cholesterol plus vitamins C+E (HCV) group. Balloon injury was induced in the right internal iliac artery 4 weeks after initiation of either dietary regimen, and plasma and tissue samples were taken at different time periods to measure PAI-1 activity and vascular inhibitor expression. The cholesterol-rich diet induced an increased in vascular PAI-1 expression in the intima, media and adventitia which was markedly reduced in the HCV group. After injury, severe structural changes were observed in NC and HC animals associated with increased systemic PAI-1 activity (P<0.001) and local PAI-1 expression being more intense in HC group. Vitamins C and E significantly reduced plasma PAI-1 activity (P=0.018) and attenuated the inhibitor expression as compared with HC.This experimental study in a porcine model of hypercholesterolemia demonstrates that vitamins C and E reduce local and systemic PAI-1 induced after angioplasty as well as the hypercholesterolemia-induced vascular PAI-1.
The importance of nail morphology in the differential diagnosis of pityriasis rubra pilaris, psoriasis and chronic erythroderma
Authors: T.S. Sonnex, R.P.R. Dawber, C.B. Zachary, P.R. Millard, W.A.D. Griffiths
Journal: British Journal of Dermatology (1984)

Journal Article The importance of nail morphology in the differential diagnosis of pityriasis rubra pilaris, psoriasis and chronic erythroderma Get access T.S. Sonnex, T.S. Sonnex St John's Hospital for Diseases of the Skin, London WC2H 7BJ Search for other works by this author on: Oxford Academic Google Scholar R.P.R. Dawber, R.P.R. Dawber The Slade and John Radeliffe Hospitals, Oxford Search for other works by this author on: Oxford Academic Google Scholar C.B. Zachary, C.B. Zachary Guy;s Hospital, London SE1 9RT Search for other works by this author on: Oxford Academic Google Scholar P.R. Millard, P.R. Millard The Slade and John Radeliffe Hospitals, Oxford Search for other works by this author on: Oxford Academic Google Scholar W.A.D. Griffiths W.A.D. Griffiths St John's Hospital for Diseases of the Skin, London WC2H 7BJ Search for other works by this author on: Oxford Academic Google Scholar British Journal of Dermatology, Volume 111, Issue s26, 1 July 1984, Pages 16–17, https://doi.org/10.1111/j.1365-2133.1984.tb05232.x Published: 01 July 1984
Is Cytology Reliable for Epidermal Growth Factor Receptor Gene Evaluation in Non-small Cell Lung Cancer?
Authors: Cecilia Bozzetti, Marcello Tiseo, Costanza Lagrasta, Rita Nizzoli, Annamaria Guazzi, Gallia Graiani, Guido Rindi, Andrea Ardizzoni
Journal: Journal of Thoracic Oncology (2010)
The Golden Years
Authors: Ronald Pies
Journal: JAMA (2005)

Our website uses cookies to enhance your experience. By continuing to use our site, or clicking "Continue," you are agreeing to our Cookie Policy | Continue JAMA HomeNew OnlineCurrent IssueFor Authors Podcasts Clinical Reviews Editors' Summary Medical News Author Interviews More Publications JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry (1919-1959) JN Learning / CMESubscribeJobsInstitutions / LibrariansReprints & Permissions Terms of Use | Privacy Policy | Accessibility Statement 2023 American Medical Association. All Rights Reserved Search All JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Forum Archive JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry Input Search Term Sign In Individual Sign In Sign inCreate an Account Access through your institution Sign In Purchase Options: Buy this article Rent this article Subscribe to the JAMA journal
A new in vivo screening model for posterior spinal bone formation: Comparison of ten calcium phosphate ceramic material treatments
Authors: Clayton E. Wilson, Moyo C. Kruyt, Joost D. de Bruijn, Clemens A. van Blitterswijk, F. Cumhur Oner, Abraham J. Verbout, Wouter J.A. Dhert
Journal: Biomaterials (2005)
INFANTILISM AND OTHER HYPOPLASTIC CONDITIONS OF THE UTERUS
Authors: EMIL NOVAK
Journal: JAMA (1918)

Infantilism of the female genital organs may be only a local manifestation of general infantilism (infantilismus universalis). More frequently, however, it is observed in women who, aside from the genital organs, present no recognizable defects in development, and who, for that matter, may be unusually well developed. It is this local form of infantilism (infantilismus partialis or genitalis) with which we are concerned in this paper. Its very great frequency can be attested by every gynecologist, although I know of no figures bearing on this point. The clinical importance of the condition rests chiefly on two considerations: (1) the frequency with which it is associated with aberrations of the menstrual function, and (2) the fact that it often seems to constitute the anatomic basis for sterility. Strictly speaking, the term "infantile uterus" should indicate that the organ resembles that normally found in the infant. The development of the uterus may,
Evaluation of Attenuation Corrected Dual Head Coincidence Imaging in Detection of Primary Pulmonary Lung Lesions
Authors: S Salem, H El-Zeftawy, A Zaher, J Santiago, S Jana, S Naddaf, J Luo, D Montanaro, H Abdel-Dayem
Journal: Clinical Nuclear Medicine (1999)

Scientific Abstracts And Posters From The 44Th Annual Meeting Of The Southwestern Chapter Of The Society Of Nuclear Medicine, And From The 12Th Northeastern Regional Scientific Meeting Of The New England And New York Chapters Of The Society Of Nuclear Medicine
INTRACRANIAL BLEEDINGS AND ISCHEMIC STROKES DURING WARFARIN TREATMENT IN PATIENTS WITH ATRIAL FIBRILLATION: THE FIBSTROKE STUDY
Authors: Antti Palomaki, Pirjo Mustonen, Tuija Vasankari, Ilpo Nuotio, Antti Ylitalo, Jussi Jaakkola, Riho Luite, Paivi Hartikainen, Juha Hartikainen, Juhani Airaksinen
Journal: Journal of the American College of Cardiology (2014)