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803 results on '"Schulz, Angela"'

151. Additional file 2: of MEST mediates the impact of prenatal bisphenol A exposure on long-term body weight development

Author

Junge, Kristin, Leppert, Beate, Jahreis, Susanne, Wissenbach, Dirk, Feltens, Ralph, Grützmann, Konrad, Thürmann, Loreen, Bauer, Tobias, Ishaque, Naveed, Schick, Matthias, Bewerunge-Hudler, Melanie, Röder, Stefan, Bauer, Mario, Schulz, Angela, Borte, Michael, Landgraf, Kathrin, Körner, Antje, Kiess, Wieland, Bergen, Martin Von, Stangl, Gabriele, Trump, Saskia, Eils, Roland, Polte, Tobias, and Lehmann, Irina

Abstract

Figure S1. Shown are the location of the MEST gene on chromosome 7 (upper part), the 450 K array CpG in the MEST promoter (middle part) and the region covered by the MassARRAY amplicon within the promoter region (CpG sites are depicted in red). (PDF 32 kb)

Published
2018
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156. Evidence of Müller Glial Dysfunction in Patients with Aquaporin-4 Immunoglobulin G–Positive Neuromyelitis Optica Spectrum Disorder

Author

You, Yuyi, primary, Zhu, Ling, additional, Zhang, Ting, additional, Shen, Ting, additional, Fontes, Ariadna, additional, Yiannikas, Con, additional, Parratt, John, additional, Barton, Joshua, additional, Schulz, Angela, additional, Gupta, Vivek, additional, Barnett, Michael H., additional, Fraser, Clare L., additional, Gillies, Mark, additional, Graham, Stuart L., additional, and Klistorner, Alexander, additional

Published
2019
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157. Lysosomal proteome analysis reveals that CLN3-defective cells have multiple enzyme deficiencies associated with changes in intracellular trafficking

Author

Schmidtke, Carolin, primary, Tiede, Stephan, additional, Thelen, Melanie, additional, Käkelä, Reijo, additional, Jabs, Sabrina, additional, Makrypidi, Georgia, additional, Sylvester, Marc, additional, Schweizer, Michaela, additional, Braren, Ingke, additional, Brocke-Ahmadinejad, Nahal, additional, Cotman, Susan L., additional, Schulz, Angela, additional, Gieselmann, Volkmar, additional, and Braulke, Thomas, additional

Published
2019
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160. Microglia actively remodels adult hippocampal neurogenesis through the phagocytosis secretome

Author

Diaz-Aparicio, Irune, primary, Paris, Iñaki, additional, Sierra-Torre, Virginia, additional, Plaza-Zabala, Ainhoa, additional, Rodríguez-Iglesias, Noelia, additional, Márquez-Ropero, Mar, additional, Beccari, Sol, additional, Abiega, Oihane, additional, Alberdi, Elena, additional, Matute, Carlos, additional, Bernales, Irantzu, additional, Schulz, Angela, additional, Otrokocsi, Lilla, additional, Sperlagh, Beata, additional, Happonen, Kaisa E., additional, Lemke, Greg, additional, Maletic-Savatic, Mirjana, additional, Valero, Jorge, additional, and Sierra, Amanda, additional

Published
2019
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161. Analysis of the genomic architecture of a complex trait locus in hypertensive rat models links Tmem63c to kidney damage

Author

Schulz, Angela, primary, Müller, Nicola Victoria, additional, van de Lest, Nina Anne, additional, Eisenreich, Andreas, additional, Schmidbauer, Martina, additional, Barysenka, Andrei, additional, Purfürst, Bettina, additional, Sporbert, Anje, additional, Lorenzen, Theodor, additional, Meyer, Alexander M, additional, Herlan, Laura, additional, Witten, Anika, additional, Rühle, Frank, additional, Zhou, Weibin, additional, de Heer, Emile, additional, Scharpfenecker, Marion, additional, Panáková, Daniela, additional, Stoll, Monika, additional, and Kreutz, Reinhold, additional

Published
2019
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162. Author response: Analysis of the genomic architecture of a complex trait locus in hypertensive rat models links Tmem63c to kidney damage

Author

Schulz, Angela, primary, Müller, Nicola Victoria, additional, van de Lest, Nina Anne, additional, Eisenreich, Andreas, additional, Schmidbauer, Martina, additional, Barysenka, Andrei, additional, Purfürst, Bettina, additional, Sporbert, Anje, additional, Lorenzen, Theodor, additional, Meyer, Alexander M, additional, Herlan, Laura, additional, Witten, Anika, additional, Rühle, Frank, additional, Zhou, Weibin, additional, de Heer, Emile, additional, Scharpfenecker, Marion, additional, Panáková, Daniela, additional, Stoll, Monika, additional, and Kreutz, Reinhold, additional

Published
2019
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166. Reduction in corpora lutea number in obese melanocortin-4-receptor-deficient mice

Author

Schöneberg Torsten, Merkwitz Claudia, Schulz Angela, Sandrock Mara, Spanel-Borowski Katharina, and Ricken Albert

Subjects
Gynecology and obstetrics, RG1-991, Reproduction, QH471-489
Abstract

Abstract Obese melanocortin-4-receptor-deficient (MC4R-/-) male mice are reported to have erectile dysfunction, while homozygous MC4R-/- female mice are apparently fertile. A recently established obese mouse strain, carrying an inactivating mutation in the MC4R gene, revealed difficulties in breeding for the homozygous female mice. This prompted us to determine the presence of follicles and corpora lutea (CL) in ovaries of MC4R-/- mice aged 3–6 months in comparison to wild type (MC4R+/+) littermates. Serial sections of formaldehyde-fixed ovaries of mice with vaginal signs of estrus and metestrus were assessed for the number of healthy and regressing follicles and CL. The number of CL, as an estimate for the ovulation rate, decreased to zero during aging in MC4R-/- mice. The number of small- (diameter 100–200 micrometer) and large-sized follicles namely antral follicles (diameter >200 micrometer) were slightly increased in MC4R-/- compared to MC4R+/+ mice. Greater differences were found in very large to cystic follicles, which were more numerous in MC4R-/- mice. The number of regressing antral follicles was higher in the MC4R-/- group compared to the MC4R+/+ group. This was associated with a wide range in the number of collapsed zonae pellucidae as the last remnants of regressed follicles. A conspicuous hypertrophy of the interstitial cells was noted in 6-month-old MC4R-/- mice. In conclusion, cystic follicles and the reduction in CL number point to a decreased ovulation rate in obese MC4R-/- mice.

Published
2009
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167. Clinical Pharmacokinetics and Pharmacodynamics of Cerliponase Alfa, Enzyme Replacement Therapy for CLN2 Disease by Intracerebroventricular Administration.

Author

Kim, Aryun, Grover, Anita, Hammon, Kevin, Hart, Greg, Slasor, Peter, Cherukuri, Anu, Ajayi, Temitayo, Jacoby, David, Schulz, Angela, Specchio, Nicola, Los Reyes, Emily, Gissen, Paul, and Henshaw, Joshua W.

Subjects
PHARMACOKINETICS, PHARMACODYNAMICS, ENZYME deficiency, ENZYMES, LYSOSOMES, NEURODEGENERATION, CEREBROSPINAL fluid, ENZYME replacement therapy
Abstract

Cerliponase alfa is recombinant human tripeptidyl peptidase 1 (TPP1) delivered by i.c.v. infusion for CLN2, a pediatric neurodegenerative disease caused by deficiency in lysosomal enzyme TPP1. We report the pharmacokinetics (PK) and pharmacodynamics of cerliponase alfa, the first i.c.v. enzyme replacement therapy, characterized in a phase I/II study. Escalating doses (30–300 mg Q2W) followed by 300 mg Q2W for ≥ 48 weeks were administered in 24 patients aged ≥ 3 years. Concentrations peaked in cerebrospinal fluid (CSF) at the end of ~ 4‐hour i.c.v. infusion and 8 hours thereafter in plasma. Plasma exposure was 300–1,000‐fold lower than in CSF, with no correlation in the magnitude of peak concentration (Cmax) or area under the concentration‐time curve (AUC) among body sites. There was no apparent accumulation in CSF or plasma exposure with Q2W dosing. Interpatient and intrapatient variability of AUC, respectively, were 31–49% and 24% in CSF vs. 59–103% and 80% in plasma. PK variability was not explained by baseline demographics, as sex, age, weight, and CLN2 disease severity score did not appear to impact CSF or plasma PK. No apparent correlation was noted between CSF or plasma PK and incidence of adverse events (pyrexia, hypersensitivity, seizure, and epilepsy) or presence of antidrug antibodies in CSF and serum. There was no relationship between magnitude of CSF exposure and efficacy (change in CLN2 score from baseline), indicating maximum benefit was obtained across the range of exposures with 300 mg Q2W. Data from this small trial of ultra‐rare disease were leveraged to adequately profile cerliponase alfa and support 300 mg i.c.v. Q2W for CLN2 treatment. [ABSTRACT FROM AUTHOR]

Published
2021
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168. Effects of cafestol and kahweol from coffee grounds on serum lipids and serum liver enzymes in humans

Author

Urgert, Rob, Schulz, Angela G.M., and Katan, Martijn B.

Subjects
Diterpenes -- Measurement, Blood cholesterol -- Measurement, Alanine aminotransferase -- Physiological aspects, Food/cooking/nutrition, Health
Abstract

The diterpenes cafestol average, NSLP lunches provide the program goal of at least one-third of the recommended dietary allowance (RDA) for most nutrients. The average amounts of total fat and saturated fat as a percentage of food energy in NSLP lunches, however, exceed the Dietary Guidelines' goals. The amount of sodium exceeds National Research Council (NRC) recommendations, but the amount of cholesterol is within the recommended range. With the exception of food energy, SBP breakfasts on average provide the program goal of at least one-fourth of the RDA. They are close to the Dietary Guidelines' goal for total fat, but exceed it for saturated fat. SBP breakfasts provide amounts of cholesterol that exceed the recommendations.

Published
1995

169. Enzymatic diagnosis of neuronal lipofuscinoses in dried blood spots using substrates for concomitant tandem mass spectrometry and fluorimetry.

Author

Maeser, Stefan, Petre, Brindusa‐Alina, Ion, Laura, Rawer, Stephan, Kohlschütter, Alfried, Santorelli, Filippo M., Simonati, Alessandro, Schulz, Angela, and Przybylski, Michael

Subjects
TANDEM mass spectrometry, PEPTIDASE, FLUORIMETRY, CATHEPSIN D, DIAGNOSIS, MASS spectrometry
Abstract

Neuronal ceroid lipofuscinoses (NCLs) are a group of neurodegenerative diseases predominantly in childhood that are characterized by psychomotor deterioration, epilepsy, and early death of patients. The NCLs analyzed in the present study are caused by defects of the specific enzymes, CLN1 (palmitoyl protein thioesterase 1; PPT1), CLN2 (tripeptidyl peptidase 1; TPP1), and CLN10 (cathepsin D). Specific and sensitive diagnostic assays of NCLs were the main goal of this study. They are of increasing importance, particularly since enzyme replacement therapy (ERT) for NCL2 has recently become available for clinical treatment, and ERTs for further NCLs are under development. Here, we report specific and sensitive determinations for CLN1, CLN2, and CLN10 on dried blood spots by tandem mass spectrometry using multiple reaction monitoring mass spectrometry (MRM‐MS). Identical substrates suitable for (i) fluorimetric determination of single enzymes and (ii) for MRM‐MS determination of multiple enzymes were synthesized by chemical coupling of alkyl‐umbelliferone building blocks with the corresponding peptidyl‐substrate groups recognized by the target enzyme. Enzymatic determinations were performed both by fluorimetry and MRM‐MS in patients with NCL1, NCL2, and NCL10 and showed good agreement in single assays. Moreover, duplex and triplex determinations were successfully performed for NCL1, NCL2, and NCL10. Specific peptidyl‐(4‐alkyl‐umbelliferone) substrates were also synthesized for mass spectrometric determinations of different cathepsins (cathepsins‐D, ‐F, and ‐B), to provide a differentiation of proteolytic specificities. [ABSTRACT FROM AUTHOR]

Published
2021
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170. Management Strategies For Cln2 Disease

Author

Williams, Ruth E., Adams, Heather R., Blohm, Martin, Cohen-Pfeffer, Jessica L., de los Reyes, Emily, Denecke, Jonas, Drago, Kristen, Fairhurst, Charlie, Frazier, Margie, Guelbert, Norberto, Kiss, Szilard, Kofler, Annamaria, Lawson, John A., Lehwald, Lenora, Leung, Mary-Anne, Mikhaylova, Svetlana, Mink, Jonathan W., Nickel, Miriam, Shediac, Renee, Sims, Katherine, Specchio, Nicola, Topcu, Meral, von Loebbecke, Ina, West, Andrea, Zernikow, Boris, Schulz, Angela, and Çocuk Sağlığı ve Hastalıkları

Subjects
Neurosciences & Neurology, Pediatrics
Abstract

CLN2 disease (neuronal ceroid lipofuscinosis type 2) is a rare, autosomal recessive, pediatric-onset, rapidly progressive neurodegenerative lysosomal storage disorder caused by tripeptidyl peptidase 1 (TPP1) enzyme deficiency, and is characterized by language delay, seizures, rapid cognitive and motor decline, blindness, and early death. No management guidelines exist and there is a paucity of published disease-specific evidence to inform clinical practice, which currently draws upon experience from the field of childhood neurodisability. Twenty-four disease experts were surveyed on CLN2 disease management and a subset met to discuss current practice. Management goals and strategies are consistent among experts globally and are guided by the principles of pediatric palliative care. Goals and interventions evolve as the disease progresses, with a shift in focus from maintenance of function early in the disease to maintenance of quality of life. A multidisciplinary approach is critical for optimal patient care. This work represents an initial step toward the development of consensus-based management guidelines for CLN2 disease.

Published
2017

172. Determination of retinal nerve fibre layer and ganglion cell/inner plexiform layers progression rates using two optical coherence tomography systems: The PROGRESSA study.

Author

Saks, Danit, Schulz, Angela, Craig, Jamie, and Graham, Stuart

Subjects
*OPTICAL coherence tomography, *GANGLIA, *NERVES, *OPTIC nerve, *FIBERS, *CHOROID, *NERVE fibers
Abstract

Importance: Glaucoma progression rates may differ depending on the retinal structural parameters measured, and between devices. Background: To compare retinal nerve fibre layer (RNFL) and ganglion cell/inner plexiform layers (GCL/IPL) progression rates using two spectral‐domain optical coherence tomography (OCT) systems. Design: Prospective, university hospital setting. Participants: Cross‐sectional study: 100 eyes from 53 glaucoma suspects and early manifest glaucoma cases. Longitudinal study: subset of 61 eyes from 33 participants. Methods: Same day optic nerve and macular images were acquired using Cirrus and Spectralis systems from which RNFL and GCL/IPL thicknesses were calculated. Longitudinal analysis of RNFL and GCL/IPL progression rates was calculated from 6 × 6‐monthly follow‐up OCT scans. Main Outcome Measures: RNFL and GCL/IPL thicknesses in matched superior, inferior and global regions were compared by both systems cross‐sectionally and longitudinally. Results: At baseline, no RNFL thicknesses differed between devices. Cirrus GCL/IPL regions were significantly thicker than Spectralis (P <.001). RNFL and GCL/IPL global progression rates (μm/y) had a mean (SD) of −1.28 (1.11) and 95% CI: (−1.48, −1.09) and −0.51 (0.58) and 95% CI: (−0.62, −0.41), respectively. Progression rates were similar across devices. RNFL loss (%) progressed significantly faster than GCL/IPL, in all regions (P ≤.004). Conclusion and Relevance: Despite baseline thickness differences, overall Cirrus and Spectralis provided similar rates of RNFL and GCL/IPL progression in early glaucoma and can be considered comparable, though not interchangeable, in clinical practice. Further analysis is needed to determine if RNFL progresses faster than GCL/IPL in glaucoma, and whether one precedes the other. [ABSTRACT FROM AUTHOR]

Published
2020
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174. An incretin-based tri-agonist promotes superior insulin secretion from murine pancreatic islets via PLC activation

Author

Khajavi, Noushafarin, primary, Finan, Brian, additional, Kluth, Oliver, additional, Müller, Timo D., additional, Mergler, Stefan, additional, Schulz, Angela, additional, Kleinau, Gunnar, additional, Scheerer, Patrick, additional, Schürmann, Annette, additional, Gudermann, Thomas, additional, Tschöp, Matthias H., additional, Krude, Heiko, additional, DiMarchi, Richard D., additional, and Biebermann, Heike, additional

Published
2018
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175. Autophagic vacuolar myopathy is a common feature of CLN3 disease

Author

Radke, Josefine, primary, Koll, Randi, additional, Gill, Esther, additional, Wiese, Lars, additional, Schulz, Angela, additional, Kohlschütter, Alfried, additional, Schuelke, Markus, additional, Hagel, Christian, additional, Stenzel, Werner, additional, and Goebel, Hans H., additional

Published
2018
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176. Finerenone Attenuates Endothelial Dysfunction and Albuminuria in a Chronic Kidney Disease Model by a Reduction in Oxidative Stress

Author

González-Blázquez, Raquel, primary, Somoza, Beatriz, additional, Gil-Ortega, Marta, additional, Martín Ramos, Miriam, additional, Ramiro-Cortijo, David, additional, Vega-Martín, Elena, additional, Schulz, Angela, additional, Ruilope, Luis Miguel, additional, Kolkhof, Peter, additional, Kreutz, Reinhold, additional, and Fernández-Alfonso, María S., additional

Published
2018
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180. A4708 Finerenone improves endothelial function through the increase in nitric oxide availability in a rat model of chronic kidney disease

Author

Fernandez-Alfonso, Maria S, primary, González-Blazquez, Raquel, additional, Vega, Elena, additional, Gil-Ortega, Marta, additional, Martin-Ramos, Miriam, additional, Ruilope, Luis Miguel, additional, Schulz, Angela, additional, Kolkhof, Peter, additional, Somoza, Beatriz, additional, and Kreutz, Reinhold, additional

Published
2018
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183. Disease characteristics and progression in patients with late-infantile neuronal ceroid lipofuscinosis type 2 (CLN2) disease: an observational cohort study

Author

Nickel, Miriam, primary, Simonati, Alessandro, additional, Jacoby, David, additional, Lezius, Susanne, additional, Kilian, Dirk, additional, Van de Graaf, Benjamin, additional, Pagovich, Odelya E, additional, Kosofsky, Barry, additional, Yohay, Kaleb, additional, Downs, Matthew, additional, Slasor, Peter, additional, Ajayi, Temitayo, additional, Crystal, Ronald G, additional, Kohlschütter, Alfried, additional, Sondhi, Dolan, additional, and Schulz, Angela, additional

Published
2018
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184. Study of Intraventricular Cerliponase Alfa for CLN2 Disease

Author

Schulz, Angela, primary, Ajayi, Temitayo, additional, Specchio, Nicola, additional, de Los Reyes, Emily, additional, Gissen, Paul, additional, Ballon, Douglas, additional, Dyke, Jonathan P., additional, Cahan, Heather, additional, Slasor, Peter, additional, Jacoby, David, additional, and Kohlschütter, Alfried, additional

Published
2018
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188. Tumor necrosis factor receptor signaling is a driver of chronic lymphocytic leukemia that can be therapeutically targeted by the flavonoid wogonin

Author

Dürr, Claudia, primary, Hanna, Bola S., additional, Schulz, Angela, additional, Lucas, Fabienne, additional, Zucknick, Manuela, additional, Benner, Axel, additional, Clear, Andrew, additional, Ohl, Sibylle, additional, Öztürk, Selcen, additional, Zenz, Thorsten, additional, Stilgenbauer, Stephan, additional, Li-Weber, Min, additional, Krammer, Peter H., additional, Gribben, John G., additional, Lichter, Peter, additional, and Seiffert, Martina, additional

Published
2018
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191. Functional lung MRI for regional monitoring of patients with cystic fibrosis

Author

Kaireit, Till F., primary, Sorrentino, Sajoscha A., additional, Renne, Julius, additional, Schoenfeld, Christian, additional, Voskrebenzev, Andreas, additional, Gutberlet, Marcel, additional, Schulz, Angela, additional, Jakob, Peter M., additional, Hansen, Gesine, additional, Wacker, Frank, additional, Welte, Tobias, additional, Tümmler, Burkhard, additional, and Vogel-Claussen, Jens, additional

Published
2017
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194. Tumor-derived exosomes modulate PD-L1 expression in monocytes

Author

Haderk, Franziska, primary, Schulz, Ralph, additional, Iskar, Murat, additional, Cid, Laura Llaó, additional, Worst, Thomas, additional, Willmund, Karolin V., additional, Schulz, Angela, additional, Warnken, Uwe, additional, Seiler, Jana, additional, Benner, Axel, additional, Nessling, Michelle, additional, Zenz, Thorsten, additional, Göbel, Maria, additional, Dürig, Jan, additional, Diederichs, Sven, additional, Paggetti, Jérôme, additional, Moussay, Etienne, additional, Stilgenbauer, Stephan, additional, Zapatka, Marc, additional, Lichter, Peter, additional, and Seiffert, Martina, additional

Published
2017
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196. Management Strategies for CLN2 Disease

Author

Williams, Ruth E., primary, Adams, Heather R., additional, Blohm, Martin, additional, Cohen-Pfeffer, Jessica L., additional, de los Reyes, Emily, additional, Denecke, Jonas, additional, Drago, Kristen, additional, Fairhurst, Charlie, additional, Frazier, Margie, additional, Guelbert, Norberto, additional, Kiss, Szilárd, additional, Kofler, Annamaria, additional, Lawson, John A., additional, Lehwald, Lenora, additional, Leung, Mary-Anne, additional, Mikhaylova, Svetlana, additional, Mink, Jonathan W., additional, Nickel, Miriam, additional, Shediac, Renée, additional, Sims, Katherine, additional, Specchio, Nicola, additional, Topcu, Meral, additional, von Löbbecke, Ina, additional, West, Andrea, additional, Zernikow, Boris, additional, and Schulz, Angela, additional

Published
2017
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197. Altered hepatic lipid metabolism in mice lacking both the melanocortin type 4 receptor and low density lipoprotein receptor

Author

Lede, Vera, primary, Meusel, Andrej, additional, Garten, Antje, additional, Popkova, Yulia, additional, Penke, Melanie, additional, Franke, Christin, additional, Ricken, Albert, additional, Schulz, Angela, additional, Kiess, Wieland, additional, Huster, Daniel, additional, Schöneberg, Torsten, additional, and Schiller, Jürgen, additional

Published
2017
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