17 results on '"W. Radner"'
Search Results
2. ABHD5—A Regulator of Lipid Metabolism Essential for Diverse Cellular Functions
- Author
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Margarita Schratter, Achim Lass, and Franz P. W. Radner
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ABHD5 ,CGI-58 ,ATGL ,PNPLA3 ,triglyceride ,lipid metabolism ,Microbiology ,QR1-502 - Abstract
The α/β-Hydrolase domain-containing protein 5 (ABHD5; also known as comparative gene identification-58, or CGI-58) is the causative gene of the Chanarin-Dorfman syndrome (CDS), a disorder mainly characterized by systemic triacylglycerol accumulation and a severe defect in skin barrier function. The clinical phenotype of CDS patients and the characterization of global and tissue-specific ABHD5-deficient mouse strains have demonstrated that ABHD5 is a crucial regulator of lipid and energy homeostasis in various tissues. Although ABHD5 lacks intrinsic hydrolase activity, it functions as a co-activating enzyme of the patatin-like phospholipase domain-containing (PNPLA) protein family that is involved in triacylglycerol and glycerophospholipid, as well as sphingolipid and retinyl ester metabolism. Moreover, ABHD5 interacts with perilipins (PLINs) and fatty acid-binding proteins (FABPs), which are important regulators of lipid homeostasis in adipose and non-adipose tissues. This review focuses on the multifaceted role of ABHD5 in modulating the function of key enzymes in lipid metabolism.
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- 2022
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3. Small-Molecule Inhibitors Targeting Lipolysis in Human Adipocytes
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Gernot F. Grabner, Nikolaus Guttenberger, Nicole Mayer, Anna K. Migglautsch-Sulzer, Christian Lembacher-Fadum, Nermeen Fawzy, Dominik Bulfon, Peter Hofer, Thomas Züllig, Lennart Hartig, Natalia Kulminskaya, Gabriel Chalhoub, Margarita Schratter, Franz P. W. Radner, Karina Preiss-Landl, Sarah Masser, Achim Lass, Rudolf Zechner, Karl Gruber, Monika Oberer, Rolf Breinbauer, and Robert Zimmermann
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Mice ,Colloid and Surface Chemistry ,Lipolysis ,Fatty Acids ,Adipocytes ,Animals ,Humans ,General Chemistry ,Biochemistry ,Catalysis ,Acyltransferases - Abstract
Chronically elevated circulating fatty acid levels promote lipid accumulation in nonadipose tissues and cause lipotoxicity. Adipose triglyceride lipase (ATGL) critically determines the release of fatty acids from white adipose tissue, and accumulating evidence suggests that inactivation of ATGL has beneficial effects on lipotoxicity-driven disorders including insulin resistance, steatohepatitis, and heart disease, classifying ATGL as a promising drug target. Here, we report on the development and biological characterization of the first small-molecule inhibitor of human ATGL. This inhibitor, designated NG-497, selectively inactivates human and nonhuman primate ATGL but not structurally and functionally related lipid hydrolases. We demonstrate that NG-497 abolishes lipolysis in human adipocytes in a dose-dependent and reversible manner. The combined analysis of mouse- and human-selective inhibitors, chimeric ATGL proteins, and homology models revealed detailed insights into enzyme-inhibitor interactions. NG-497 binds ATGL within a hydrophobic cavity near the active site. Therein, three amino acid residues determine inhibitor efficacy and species selectivity and thus provide the molecular scaffold for selective inhibition.
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- 2022
4. Correction: Mutations in Cause Autosomal Recessive Congenital Ichthyosis in Humans.
- Author
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Franz P. W. Radner, Slaheddine Marrakchi, Peter Kirchmeier, Gwang-Jin Kim, Florence Ribierre, Bourane Kamoun, Leila Abid, Michael Leipoldt, Hamida Turki, Werner Schempp, Roland Heilig, Mark Lathrop, and Judith Fischer
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Genetics ,QH426-470 - Published
- 2013
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5. Mutations in CERS3 cause autosomal recessive congenital ichthyosis in humans.
- Author
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Franz P W Radner, Slaheddine Marrakchi, Peter Kirchmeier, Gwang-Jin Kim, Florence Ribierre, Bourane Kamoun, Leila Abid, Michael Leipoldt, Hamida Turki, Werner Schempp, Roland Heilig, Mark Lathrop, and Judith Fischer
- Subjects
Genetics ,QH426-470 - Abstract
Autosomal recessive congenital ichthyosis (ARCI) is a rare genetic disorder of the skin characterized by abnormal desquamation over the whole body. In this study we report four patients from three consanguineous Tunisian families with skin, eye, heart, and skeletal anomalies, who harbor a homozygous contiguous gene deletion syndrome on chromosome 15q26.3. Genome-wide SNP-genotyping revealed a homozygous region in all affected individuals, including the same microdeletion that partially affects two coding genes (ADAMTS17, CERS3) and abolishes a sequence for a long non-coding RNA (FLJ42289). Whereas mutations in ADAMTS17 have recently been identified in autosomal recessive Weill-Marchesani-like syndrome in humans and dogs presenting with ophthalmologic, cardiac, and skeletal abnormalities, no disease associations have been described for CERS3 (ceramide synthase 3) and FLJ42289 so far. However, analysis of additional patients with non-syndromic ARCI revealed a splice site mutation in CERS3 indicating that a defect in ceramide synthesis is causative for the present skin phenotype of our patients. Functional analysis of patient skin and in vitro differentiated keratinocytes demonstrated that mutations in CERS3 lead to a disturbed sphingolipid profile with reduced levels of epidermis-specific very long-chain ceramides that interferes with epidermal differentiation. Taken together, these data present a novel pathway involved in ARCI development and, moreover, provide the first evidence that CERS3 plays an essential role in human sphingolipid metabolism for the maintenance of epidermal lipid homeostasis.
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- 2013
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6. Triage of patients with acute diverticulitis: are some inpatients candidates for outpatient treatment?
- Author
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Raoul J. Burchette, Rebecca R. Cannom, Vicki Y. Chiu, David A. Etzioni, Maher A. Abbas, G. W. Radner, and Philip I. Haigh
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Male ,medicine.medical_specialty ,Abdominal Abscess ,Percutaneous ,Decision Making ,Diverticulitis, Colonic ,Ambulatory care ,Statistical significance ,Ambulatory Care ,medicine ,Humans ,Abscess ,Aged ,Retrospective Studies ,First episode ,Analysis of Variance ,business.industry ,Age Factors ,Gastroenterology ,Retrospective cohort study ,Length of Stay ,Middle Aged ,Diverticulitis ,medicine.disease ,Triage ,Surgery ,Acute Disease ,Emergency medicine ,Drainage ,Female ,Tomography, X-Ray Computed ,business - Abstract
Aim Current recommendations regarding the triage of patients with acute diverticulitis for inpatient or outpatient treatment are vague. We hypothesized that a significant number of patients treated as an inpatient could be managed as an outpatient. Method A retrospective cohort study was carried out of 639 patients admitted for a first episode of diverticulitis. The diagnosis of acute diverticulitis was confirmed by computed tomography (CT). The endpoints included length of stay, need for surgery, percutaneous drainage and mortality. Patients were considered to have had a minimal hospitalization, defined as survival to discharge without needing a procedure, hospitalization of ≤ 3 days and no readmission for diverticulitis within 30 days after discharge. Results Of 639 patients, 368 (57.6%) had a minimal hospitalization. Female gender and CT scan findings of free air/fluid were negatively associated with the likelihood of minimal hospitalization. The presence of an abscess
- Published
- 2013
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7. Monoglyceride lipase deficiency causes desensitization of intestinal cannabinoid receptor type 1 and increased colonic μ-opioid receptor sensitivity
- Author
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U, Taschler, T O, Eichmann, F P W, Radner, G F, Grabner, H, Wolinski, M, Storr, A, Lass, R, Schicho, and R, Zimmermann
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Male ,Mice, Knockout ,Cannabinoids ,Colon ,digestive, oral, and skin physiology ,Receptors, Opioid, mu ,Asialoglycoproteins ,Membrane Proteins ,Research Papers ,Mice, Inbred C57BL ,Mice ,Organ Culture Techniques ,Receptor, Cannabinoid, CB1 ,Ileum ,Animals ,lipids (amino acids, peptides, and proteins) ,Lectins, C-Type ,Gastrointestinal Motility - Abstract
Background and Purpose Monoglyceride lipase (MGL) degrades 2-arachidonoyl glycerol (2-AG), an endogenous agonist of cannabinoid receptors (CB1/2). Because the CB1 receptor is involved in the control of gut function, we investigated the effects of pharmacological inhibition and genetic deletion of MGL on intestinal motility. Furthermore, we determined whether defective 2-AG degradation affects μ-opioid receptor (μ receptor) signalling, a parallel pathway regulating gut motility. Experimental Approach Gut motility was investigated by monitoring Evans Blue transit and colonic bead propulsion in response to MGL inhibition and CB1 receptor or μ receptor stimulation. Ileal contractility was investigated by electrical field stimulation. CB1 receptor expression in ileum and colon was assessed by immunohistochemical analyses. Key Results Pharmacological inhibition of MGL slowed down whole gut transit in a CB1 receptor-dependent manner. Conversely, genetic deletion of MGL did not affect gut transit despite increased 2-AG levels. Notably, MGL deficiency caused complete insensitivity to CB1 receptor agonist-mediated inhibition of whole gut transit and ileal contractility suggesting local desensitization of CB1 receptors. Accordingly, immunohistochemical analyses of myenteric ganglia of MGL-deficient mice revealed that CB1 receptors were trapped in endocytic vesicles. Finally, MGL-deficient mice displayed accelerated colonic propulsion and were hypersensitive to μ receptor agonist-mediated inhibition of colonic motility. This phenotype was reproduced by chronic pharmacological inhibition of MGL. Conclusion and Implications Constantly elevated 2-AG levels induce severe desensitization of intestinal CB1 receptors and increased sensitivity to μ receptor-mediated inhibition of colonic motility. These changes should be considered when cannabinoid-based drugs are used in the therapy of gastrointestinal diseases.
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- 2015
8. English sentence optotypes for measuring reading acuity and speed--the English version of the Radner Reading Charts
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W. Radner and Gabriela Diendorfer
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Adult ,Male ,Vocabulary ,media_common.quotation_subject ,Visual Acuity ,Validity ,computer.software_genre ,Cellular and Molecular Neuroscience ,Young Adult ,Cronbach's alpha ,Reading (process) ,Humans ,Vision test ,media_common ,Mathematics ,Vision, Binocular ,business.industry ,Vision Tests ,Middle Aged ,Sensory Systems ,Ophthalmology ,Reading ,Female ,Artificial intelligence ,Paragraph ,business ,computer ,Words per minute ,Sentence ,Natural language processing - Abstract
To develop 28 short sentence optotypes for the English version of the Radner Reading Charts that are as comparable as possible in number and length of words, as well as in difficulty and syntactical construction. Thirty-four English sentences were constructed following the method used for other Radner Reading Charts to obtain “sentence optotypes” with comparable structure and the same lexical and grammatical difficulty. Best comparable sentences were statistically selected and standardized in 50 volunteers. Reading speed and the number of errors were determined. Validity was analyzed with a 124-word long 4th-grade paragraph of the Florida Comprehensive Assessment Test®. Computerized measurements of reading parameters were performed with the RADNER Reading Device (RAD-RD©; in conjunction with a PC and microphone). The mean reading speed obtained with the 28 selected short sentences was 201.53 ± 35.88 words per minute (wpm), as compared to 215.01 ± 30.37 wpm for the long paragraph. The mean reading times were 4.30 ± 0.79 s and 35.26 ± 4.85 s, respectively. The mean number of reading errors was 0.11 ± 0.34. The correlation between the short sentences and the long paragraph was high (r = 0.76; p
- Published
- 2014
9. Ein neues visuell-akustisch gestütztes Computeranalyseverfahren — zur exakten Messung der Lesegeschwindigkeit und — zur erleichterten Datenverwaltung bei der Bestimmung des Lesevermögens
- Author
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W. Radner
- Subjects
Gynecology ,Ophthalmology ,medicine.medical_specialty ,medicine ,Psychology - Published
- 2000
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10. Die Auswirkung der Inzisionsbreite auf das Gewebetrauma an der Hornhaut nach Implantation einer faltbaren AMO PhacoFlex II®
- Author
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Rupert Menapace, Rudolf Mallinger, W. Radner, and Martin Zehetmayer
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Gynecology ,Corneal Trauma ,Ophthalmology ,medicine.medical_specialty ,business.industry ,medicine ,business - Abstract
Durch die Entwicklung der „Clear-Cornea-Incision“ (CCI) konnte bei Kataraktoperationen das Gewebetrauma am Auge reduziert und somit eine schnelle visuelle Rehabilitation erreicht werden. Fur die CCI stehen unterschiedlich geschliffene Inzisionslanzen verschiedener Breiten zur Verfugung. Es ist jedoch bekannt, wie sich unterschiedliche Inzisionsbreiten auf das Hornhautgewebe bei Implantation einer faltbaren Intraokularlinse (IOL) auswirken. Daher untersuchten wir dies mittels Licht-(LM) und Rasterelektronenmikroskopie (REM).
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- 1997
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11. Rehabilitation sehbehinderter Personen im Erwachsenenalter
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W. Radner and N. Ségur-Eltz
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Ophthalmology ,medicine.medical_specialty ,Physical therapy ,medicine ,Psychology - Published
- 1997
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12. Zur Anordnung der kollagenen Lamellen beim Keratokonus
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Rudolf Mallinger, Christian Skorpik, W. Radner, and Martin Zehetmayer
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Ophthalmology - Abstract
Die Pathogenese des Keratokonus ist bis heute nicht geklart. So findet sich neben einer normalen Verteilung der Kollagentypen auch eine normale posttranslationale Modifikation des Kollagens. Biochemische Untersuchungen weisen beim Keratokonus jedoch auf Veranderungen der Grundsubstanz hin. Zudem unterscheidet sich das biomechanische Verhalten gegenuber Zug-, Druck- und Scherkraften von jenem der normalen Hornhaut.
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- 1996
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13. Computerunterstützte Diagnose und Therapie in der visuellen Frühförderung
- Author
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S. Priglinger and W. Radner
- Subjects
Ophthalmology - Abstract
Bei schwerst sehbehinderten Kindern sind ubliche Methoden zur Bestimmung des Sehvermogens oft nicht durchfuhrbar. Die vorliegende Arbeit beschreibt ein an unserem Hause in Zusammenarbeit mit der Firma „Techsoft“ entwickeltes Computersystem. Dieses dient der Diagnose des Sehvermogens sowie der Therapieunterstutzung bei Kindern mit visuellen Wahrnehmungsstorungen. Fur die Diagnose stehen verschiedene Testprogramme zur Verfugung: Stehende Streifen, bewegte Streifen, geometrische Objekte unterschiedlicher Grose, Form und Farbe, Preferential-Looking und Konturenbilder, wie das Mondgesicht. Der Computer ermoglicht die Anderung fast jedes einzelnen Testparameters, sowie eine Verknupfung der verschiedenen Testprogramme. Durch Abspeichern eines an die Moglichkeiten der Kinder angepasten Therapieprogrammes kann das Computersystem auch therapeutisch eingesetzt werden. Eine spezielle Tastatur ermoglicht es dem Kind aktiv in den Therapieablauf einzugreifen. Mit diesem Computersystem last sich das Sehvermogen auch bei schwerst sehbehinderten Kindern definiert und reproduzierbar dokumentieren. Wir erhoffen uns davon einen gunstigen Therapieeffekt, weil durch eine umfassende Diagnose, als standiger Begleiter der Therapie, die Kinder gezielter gefordert werden konnen. The present paper introduces into a computersystem, which was developed in cooperation with the “Firma Techsoft“. This Computersystem improves diagnosis of visual acuity as well as the therapy in children with an impaired visual perception. For documentation of the visual faculties different testprograms can be used: motionless stripes, moving stripes, geometrical objects of different size and colour, preferential-looking and outline drawings as, e.g., the moon face. The Computersystem makes it possible to change nearly all testparameters and to link testprograms together. In addition, the computer is helpful in therapy. After storage of a therapyprogram fitting to the visual faculties of these children, this program can be used for individual visual stimulation. A special keyboard gives the children the possibility to participate actively in the therapy. Defined and reproducible data about the visual faculties of children with an impaired visual perception can be drawn with this computersystem. To our mind, this should lead to favourable effects on the therapy.
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- 1993
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14. Zur dreidimensionalen Anordnung der kollagenen Lamellen im posterioren Stroma der menschlichen Hornhaut
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W. Radner, Martin Zehetmayer, Rudolf Mallinger, and Werner Kulnig
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Ophthalmology - Abstract
Die dreidimensionale Anordnung der kollagenen Lamellen in der menschlichen Hornhaut ist bis heute nicht restlos geklart. In einer Vorversuchsreihe haben wir eine spezielle Praparationstechnik zur optimalen Darstellung des Kollagens in der Cornea entwickelt und untersuchten mittels Raster- und Transmissionselektronenmikroskopie die Anordnung der subendothelialen Lamellen in verschiedenen Regionen (limbal, parazentral, zentral) der menschlichen Hornhaut. Die kollagenen Lamellen verlaufen nicht nur in einer Ebene, sondern erreichen, indem sie sich aufteilen und durchkreuzen, auch benachbarte Hornhautschichten. Die Teilungswinkel der Lamellen betragen in der gesamten Cornea etwa 25 °. Auffallig ist, das die Kreuzungswinkel in den verschiedenen Regionen unterschiedlich sind. Sie betragen in der limbalen Region durchschnittlich 25 °; parazentral und zentral variieren sie zwischen 40° und 75°. Eine limbusparallele Hauptverlaufsrichtung last sich fur die limbale und parazentrale Region ermitteln. The discussion about the three-dimensional arrangement of collagen lamellae in the human cornea is equivocal. In the present study we investigated the limbal, paracentral and central region of the posterior layer of human corneal stroma by scanning and transmission electron microscopy. In all regions lamellae split into branches at angles of about 25 ° and are interlaced by crossing each other. By doing so lamellae reach different layers. In all regions we found splitting angles of about 25 °. However, different angles of criss-crossing were found — in the limbal region of about 25 °; in the paracentral and central cornea the angles vary between 40 ° and 75 °. In the limbal and paracentral region we could demonstrate a main course of the lamellae which is parallel to the limbus.
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- 1993
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15. Ist die Lasertherapie eines diabetischen Makulaödems bei Visus 0,1 oder weniger sinnvoll?
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Eva Ries, W. Obermayer, W. Radner, A. Wedrich, and Katharina Krepler
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Ophthalmology ,medicine.medical_specialty ,business.industry ,medicine ,business - Abstract
Die Photokoagulation als Therapie eines diabetischen Makulaodems ist seit langer Zeit etabliert. Nach den Studien der ETDRS wird diese Behandlung bei Augen mit einem Visus von mindestens 0,1 oder besser empfohlen. Diese Pilotstudie untersucht die Ergebnisse einer Laserbehandlung bei Augen mit diabetischen CSME und einem Visus von 0,1 und darunter. 46 Patienten (57 Augen) wurden nach Diabetestyp, Diabetesdauer, HbA1C-Status, internistischer Therapie, Nuchternblutzucker, Nikotinabusus, Fern- und Nahvisus, Tonometrie, Katarakt und Rubeosis untersucht. Alle wiesen einen Visus von 0,1 oder weniger zu Untersuchungsbeginn auf. Nach den Beurteilungskriterien der „Airlie House Classification“ wurden die Patienten der Lasertherapie zugefuhrt. Ein fokales Makulaodem wurde mit fokal gesetzten Laserspots behandelt, wahrend ein diffuses Makulaodem mit grid Laserbehandlung therapiert wurde. Die Sehscharfemessung erfolgte alle 3–4 Monate uber insgesamt 3 Jahre, um die Entwicklung der Sehscharfe unter Laserwirkung zu beobachten. Unter den 31 Frauen und 15 Mannern war ein Typ-I-Diabetiker. Das Durchschnittsalter betrug 67 Jahre. Bei 49 (86%) Augen war der Visus stabil geblieben. In 2 Fallen (3,5%) sank die Sehscharfe auf einen quantitativen Messwert. 6 (10,5%) Augen erfuhren eine Verbesserung des Visus. Der HbA1C-Wert lag bei den Patienten mit stabilen Visus unter 9,5%, wahrend er bei den beiden mit Visusabnahme uber 11 % oder mehr lag. Bei jenen Augen, die eine Verbesserung nach Laserbehandlung aufwiesen, lag der HbA1C-Wert auffallend unter 8%. Die Laserbehandlung ist bei diabetisch bedingten CSME auch bei sehr geringer Sehleistung erwagungswert. Der Erhalt des Visus bei 0,1 uber einen mehrjahrigen Zeitraum muss als Erfolg gesehen werden. Durch bestmogliche Compliance, und dadurch erzielte Stoffwechseleinstellung, kann der Lasererfolg optimiert und dem Patienten die geringe Sehleistung erhalten werden. Photocoagulation of diabetic macular edema is established for a long time. Although this therapy is suggested by the ETDRS for eyes above vision of 0,1 or better more, this pilot study underlines the effect of laser treatment in eyes with a visual acuity of 0,1 and less. 46 patients (57 eyes) were examined about type nicotine-abusus, distance + near vision, tonometry, cataract and rubeosis. At the begin of the study all patients had a vision about 0,1 of less and all were treated by laser as it is shown by the „Airlie House Classification“. Visual acuity was tested all 3 to 4 months and continued over 3 years to observe the effectivness. Among 31 women and 15 men was one type-I-diabetic. Mean age was 67 years. In 49 (86%) eyes visual acuity was unchanged. In 2 (3,5%) eyes visual acuity reduced to handmovement. In 6 (10,5%) eyes visual acuity increased. In patients with stable vision the HbA1C-value was below 9,5%. Patients with reduced visual acuity had HbA1C-value of 11% or more. In eyes with increased visual acuity after laser treatment the HbA1C-value was remarkable below 8%. Laser treatment in eyes with diabetic macular edema and visual acuity of 0,1 or less is indicated. A visual acuity about 0,1 over years has to be defined as a success. Good bloodglucose-metabolism and compliance of patients are the most important factors for an optimal result of laser treatment and salvation of visual acuity.
- Published
- 2000
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16. Bestimmung des Sehvermögens bei schwerst seh- und mehrfachbehinderten Kindern mit dem Computer
- Author
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A. Hajek, S. Priglinger, and W. Radner
- Subjects
Ophthalmology - Abstract
Fur die gezielte visuelle Forderung von schwerst seh- und mehrfachbehinderten Kindern ist neben einer organisch-funktionellen Diagnose (CT, VEP, Fundus-skopie, Skiaskopie, Orthoptik) auch eine Bestimmung des Sehvermogens wunschenswert. Ubliche nonverbale Prufme-thoden stellen jedoch Minimalanforderungen an das visuelle System, die von diesen Kindern oft nicht erbracht werden konnen. Mit unserem Computersystem konnen wir bei schwerst seh- und mehrfachbehinderten Kindern auch dann eine definierte und reproduzierbare Information uber das Sehvermogen erhalten, wenn ubliche Methoden keine sichere Beurteilung zulassen.
- Published
- 1995
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17. Evaluating reading acuity and speed in children with microstrabismic amblyopia using a standardized reading chart system.
- Author
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E. Stifter, G. Burggasser, E. Hirmann, A. Thaler, and W. Radner
- Published
- 2005
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