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738 results on '"Mandel, A. M."'

3. In planta expression of human polyQ-expanded huntingtin fragment reveals mechanisms to prevent disease-related protein aggregation

Author

Llamas, Ernesto, Koyuncu, Seda, Lee, Hyun Ju, Wehrmann, Markus, Gutierrez-Garcia, Ricardo, Dunken, Nick, Charura, Nyasha, Torres-Montilla, Salvador, Schlimgen, Elena, Mandel, Amrei M., Theile, Erik Boelen, Grossbach, Jan, Wagle, Prerana, Lackmann, Jan-Wilm, Schermer, Bernhard, Benzing, Thomas, Beyer, Andreas, Pulido, Pablo, Rodriguez-Concepcion, Manuel, Zuccaro, Alga, and Vilchez, David

Published
2023
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5. Biocompatible metal–organic frameworks for the storage and therapeutic delivery of hydrogen sulfide

Author

Chen, Faith E, Mandel, Ruth M, Woods, Joshua J, Lee, Jung-Hoon, Kim, Jaehwan, Hsu, Jesse H, Fuentes-Rivera, José J, Wilson, Justin J, and Milner, Phillip J

Subjects
Chemical Sciences
Abstract

Hydrogen sulfide (H2S) is an endogenous gasotransmitter with potential therapeutic value for treating a range of disorders, such as ischemia-reperfusion injury resulting from a myocardial infarction or stroke. However, the medicinal delivery of H2S is hindered by its corrosive and toxic nature. In addition, small molecule H2S donors often generate other reactive and sulfur-containing species upon H2S release, leading to unwanted side effects. Here, we demonstrate that H2S release from biocompatible porous solids, namely metal-organic frameworks (MOFs), is a promising alternative strategy for H2S delivery under physiologically relevant conditions. In particular, through gas adsorption measurements and density functional theory calculations we establish that H2S binds strongly and reversibly within the tetrahedral pockets of the fumaric acid-derived framework MOF-801 and the mesaconic acid-derived framework Zr-mes, as well as the new itaconic acid-derived framework CORN-MOF-2. These features make all three frameworks among the best materials identified to date for the capture, storage, and delivery of H2S. In addition, these frameworks are non-toxic to HeLa cells and capable of releasing H2S under aqueous conditions, as confirmed by fluorescence assays. Last, a cellular ischemia-reperfusion injury model using H9c2 rat cardiomyoblast cells corroborates that H2S-loaded MOF-801 is capable of mitigating hypoxia-reoxygenation injury, likely due to the release of H2S. Overall, our findings suggest that H2S-loaded MOFs represent a new family of easily-handled solid sources of H2S that merit further investigation as therapeutic agents. In addition, our findings add Zr-mes and CORN-MOF-2 to the growing lexicon of biocompatible MOFs suitable for drug delivery.

Published
2021

6. The carboxy‐terminus of the human ARPKD protein fibrocystin can control STAT3 signalling by regulating SRC‐activation

Author

Dafinger, Claudia, Mandel, Amrei M, Braun, Alina, Göbel, Heike, Burgmaier, Kathrin, Massella, Laura, Mastrangelo, Antonio, Dötsch, Jörg, Benzing, Thomas, Weimbs, Thomas, Schermer, Bernhard, and Liebau, Max C

Subjects
Biochemistry and Cell Biology, Biological Sciences, Kidney Disease, Genetics, Polycystic Kidney Disease, Congenital Structural Anomalies, Pediatric, 2.1 Biological and endogenous factors, Aetiology, Renal and urogenital, Cell Line, Humans, Immunohistochemistry, Phosphorylation, Polycystic Kidney, Autosomal Recessive, Protein Interaction Domains and Motifs, Receptors, Cell Surface, STAT3 Transcription Factor, Signal Transduction, src-Family Kinases, cilia, genetic kidney diseases, polycystic kidney disease, Medicinal and Biomolecular Chemistry, Clinical Sciences, Biochemistry & Molecular Biology, Biochemistry and cell biology, Medicinal and biomolecular chemistry
Abstract

Autosomal recessive polycystic kidney disease (ARPKD) is mainly caused by variants in the PKHD1 gene, encoding fibrocystin (FC), a large transmembrane protein of incompletely understood cellular function. Here, we show that a C-terminal fragment of human FC can suppress a signalling module of the kinase SRC and signal transducer and activator of transcription 3 (STAT3). Consistently, we identified truncating genetic variants specifically affecting the cytoplasmic tail in ARPKD patients, found SRC and the cytoplasmic tail of fibrocystin in a joint dynamic protein complex and observed increased activation of both SRC and STAT3 in cyst-lining renal epithelial cells of ARPKD patients.

Published
2020

10. Induced crystallization method of rapid metal melts 3d-printing

Author

Oshurko, V. B., Lednev, V. N., Mandel, A. M., and Solomakho, K.

Subjects
Physics - Applied Physics, Condensed Matter - Materials Science
Abstract

A solution for the most important problems in 3D printing technology (long print times, small build volumes and limited material properties) is proposed. Method is based on the fact that temperature of melts crystallization can be shifted up by pressure. Required pressure can be created by Ampere force caused by electric current in the melt flow. Magnetohydrodynamical model (with heat taken into account) is developed. It has been shown that additional heating of melt by this current can be overcome by appropriate choice of tunable parameters and introduction of some additional magnetic field. Range of parameters where this induced crystallization can take place is found.

Published
2018

11. An upper limit on fermion mass spectrum in non-Hermitian models and its implications for studying of dark matter

Author

Rodionov, V. N. and Mandel, A. M.

Subjects
High Energy Physics - Phenomenology
Abstract

The paper formulates a principal positions of non-Hermitian models with $\gamma_5$-mass extensions, which often be ignored in some investigations for this subject. In fact in this case Hamiltonians contain not only Hermitian masses $m_1$, but also contribution from anti-Hermitian components of fermion masses $m_2$. Main misunderstanding a number of papers is consist in using of this model for any values of fermion masses for fixed values of $m_1$ and $m_2$. However the basis appearing of two parameters masses may be undertaken a simple estimation for determination maximal permissible value of fermion mass $M={m_1}^2/2m_2$ which may be used for this model. Easy to see that $M$ becomes infinite and hence experimentally does not observable only in Dirac's limit, when the non-Hermitian mass fully is disappearing. In particular the equality $m_1=m_2$ can be realized only in two cases when $m_1=m_2=0$ and $m_1=m_2=2M$. Moreover in the second case the question is about the possibility existence a number of new fermions masses which are equal to the masses of particles Standard Model(SM) but when they have the non-Hermitian characteristics. In this case the paradox of the "two masses" takes place and its solution may be done only in suggested model with a maximal mass. Appearing particles can be considered as some new particles arising beyond SM. The unusual properties of these particles allow also to consider their as possible candidates in structure of dark matter., Comment: 20 pages, 5 esp figures

Published
2017

12. Gas Delivery Relevant to Human Health using Porous Materials.

Author

Mandel, Ruth M., Lotlikar, Piyusha S., Keasler, Kaitlyn T., Chen, Elena Y., Wilson, Justin J., and Milner, Phillip J.

Subjects
*POROUS materials, *MAGNETIC resonance imaging, *METAL-organic frameworks, *ORGANIC synthesis, *FLUORINATION, *ZEOLITES
Abstract

Gases are essential for various applications relevant to human health, including in medicine, biomedical imaging, and pharmaceutical synthesis. However, gases are significantly more challenging to safely handle than liquids and solids. Herein, we review the use of porous materials, such as metal‐organic frameworks (MOFs), zeolites, and silicas, to adsorb medicinally relevant gases and facilitate their handling as solids. Specific topics include the use of MOFs and zeolites to deliver H2S for therapeutic applications, 129Xe for magnetic resonance imaging, O2 for the treatment of cancer and hypoxia, and various gases for use in organic synthesis. This Perspective aims to bring together the organic, inorganic, medicinal, and materials chemistry communities to inspire the design of next‐generation porous materials for the storage and delivery of medicinally relevant gases. [ABSTRACT FROM AUTHOR]

Published
2024
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18. Vignette of a Child with Developmental Regression, Seizures, and Combined Disorders of Movement.

Author

Radmard, Sara, Bassetti, Jennifer A., Mandel, Arthur M., and Katus, Linn

Subjects
SYMPTOMS, GENETIC disorders, GAUCHER'S disease, HUNTINGTON disease, INFORMED consent (Medical law), CEREBELLUM degeneration, SPINOCEREBELLAR ataxia
Abstract

This article presents a case study of a 14-year-old boy who exhibits developmental regression, seizures, and combined movement disorders. The boy had a history of global developmental delay and required special education. Over time, his motor function deteriorated, and he developed seizures, dysphagia, self-harm, and aggression. MRI brain scans revealed marked caudate and putamen atrophy and gliosis. The examination showed various movement abnormalities, including dystonia, Parkinsonism, and myoclonus. The differential diagnosis narrowed down to Niemann-Pick type C, Wilson's disease, pediatric Huntington's disease, and Gaucher's disease. Genetic testing confirmed pediatric Huntington's disease, which typically presents with Parkinsonism, dystonia, gait difficulties, and epilepsy. The article highlights the need for separate evaluation and prediction analyses for the pediatric population to better understand the disease progression and implement preventive measures. [Extracted from the article]

Published
2024
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20. An effective cavity resonance model for enhanced optical transmission through arrays of subwavelength apertures in metal films

Author

Lansey, Eli, Mandel, Isroel M., Gollub, Jonah N., and Crouse, David T.

Subjects
Physics - Optics
Abstract

We present a novel theoretical approach for modeling the resonant properties of transmission through subwavelength apertures penetrating metal films. We show that cavity mode theory applies to an effective resonant cavity whose dimensions are determined by the aperture's geometry and the evanescent decay lengths of the associated diffracted waves. This method suggests a concrete physical mechanism for the enhanced transmission observed in periodic aperture arrays, namely it is the evanescently scattered light, localized in the near field of metal surface, which couples into the apertures. Furthermore, it analytically predicts the frequencies of peaks in enhanced transmission, the quality factor of the peaks, and explains their dependence on variation in the hole radius, periodicity, and the film thickness over a wide range of geometries. This model demonstrates strong correlation to simulation and existing results with a high degree of accuracy., Comment: Submitted to JOSA B. Under review

Published
2013

26. On Influence of Intensive Stationary Electromagnetic Field on the Behavior of Fermionic Systems

Author

Rodionov, V. N., Mandel, A. M., and Arbuzova, E. V.

Subjects
High Energy Physics - Phenomenology, High Energy Physics - Theory
Abstract

Exact solutions of Schroedinger and Pauli equations for charged particles in an external stationary electromagnetic field of an arbitrary configuration are constructed. Green functions of scalar and spinor particles are calculated in this field. The corresponding equations for complex energy of particles bounded by short range potential are deduced. Boundary condition typical for delta - potential is not used in the treatment. Explicit analytical expressions are given for the shift and width of a quasistationary level for different configurations of the external field. The critical value of electric field in which the idea of quasistationary level becomes meaningless is calculated. It is shown that the common view on the stabilizing role of magnetic field concerns only scalar particles., Comment: 15 pages, no figures, LaTeX2e

Published
2005
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32. The role of the FSGS disease gene product and nuclear pore protein NUP205 in regulating nuclear localization and activity of transcriptional regulators YAP and TAZ

Author

Ester, Lioba, primary, Cabrita, Inês, additional, Ventzke, Michel, additional, Kieckhöfer, Emilia, additional, Christodoulou, Marita, additional, Mandel, Amrei M, additional, Diefenhardt, Paul, additional, Fabretti, Francesca, additional, Benzing, Thomas, additional, Habbig, Sandra, additional, and Schermer, Bernhard, additional

Published
2023
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46. Laser ablation is effective for temporal lobe epilepsy with and without mesial temporal sclerosis if hippocampal seizure onsets are localized by stereoelectroencephalography

Author

Youngerman, Brett E., Oh, Justin Y., Anbarasan, Deepti, Billakota, Santoshi, Casadei, Camilla H., Corrigan, Emily K., Banks, Garret P., Pack, Alison M., Choi, Hyunmi, Bazil, Carl W., Srinivasan, Shraddha, Bateman, Lisa M., Schevon, Catherine A., Feldstein, Neil A., Sheth, Sameer A., McKhann, Guy M., II, Akman, Cigdem I., Bell, Michelle W., Cole, Jeffrey, Hamberger, Marla J., Kent, Paul F., Krish, Sonia N., Levy, Kirk J., Mandel, Arthur M., and Mendiratta, Anil

Published
2018
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