Cabp2-Gene Therapy Restores Inner Hair Cell Calcium Currents and Improves Hearing in a DFNB93 Mouse Model

Autor: David Oestreicher, Maria Magdalena Picher, Vladan Rankovic, Tobias Moser, Tina Pangrsic

Publikováno v: Frontiers in Molecular Neuroscience, Vol 14 (2021)

Clinical management of auditory synaptopathies like other genetic hearing disorders is currently limited to the use of hearing aids or cochlear implants. However, future gene therapy promises restoration of hearing in selected forms of monogenic hear

Externí odkaz: https://doaj.org/article/daecb6e68fa44f6a92af0de315b24d54

The synaptic ribbon is critical for sound encoding at high rates and with temporal precision

Autor: Rituparna Chakrabarti, Maria Magdalena Picher, Susann Michanski, Carolin Wichmann, Jakob Neef, Chad P. Grabner, Mehmet Gültas, Lina María Jaime Tobón, Andreas Neef, Stephan Maxeiner, David Lopez de la Morena, Tobias Moser, SangYong Jung, Philippe Jean, Nicola Strenzke

Publikováno v: eLife
eLife, Vol 7 (2018)

We studied the role of the synaptic ribbon for sound encoding at the synapses between inner hair cells (IHCs) and spiral ganglion neurons (SGNs) in mice lacking RIBEYE (RBEKO/KO). Electron and immunofluorescence microscopy revealed a lack of synaptic

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9be8056186ac80d47fd33a6181ae79e7
https://hdl.handle.net/21.11116/0000-0000-28E3-321.11116/0000-0000-62C4-421.11116/0000-0000-62C5-3

Ca2+-binding protein 2 inhibits Ca2+-channel inactivation in mouse inner hair cells

Autor: Sandra Meese, Nicola Strenzke, Guy Van Camp, Alberto Giulio Dragonetti, Friederike Predoehl, Sang Yong Jung, Tobias Moser, Roberto Colombo, Anna Gehrt, Aleksandra Ivanovic, Maria Magdalena Picher, Isabelle Schrauwen

Publikováno v: Proceedings of the National Academy of Sciences of the United States of America

Significance Ca 2+ channels mediate excitation-secretion coupling and show little inactivation at sensory ribbon synapses, enabling reliable synaptic information transfer during sustained stimulation. Studies of Ca 2+ -channel complexes in HEK293 cel

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::6ef415eb952775593880b1fcad761196
https://hdl.handle.net/11858/00-001M-0000-002D-7096-511858/00-001M-0000-002D-7094-9

A Mutation in CABP2, Expressed in Cochlear Hair Cells, Causes Autosomal-Recessive Hearing Impairment

Autor: Guy Van Camp, Friederike Predoehl, Annelies Dheedene, Erik Fransen, Celia Zazo Seco, Amy S. Lee, Akira Inagaki, Paul Coucke, Morteza Hashemzadeh Chaleshtori, Manou Sommen, Charlotte Claes, Tobias Moser, Hannie Kremer, Mohammad Amin Tabatabaiefar, Maria Magdalena Picher, Isabelle Schrauwen, Jaap Oostrik, Sarah Helfmann

Publikováno v: American Journal of Human Genetics, 91, 636-45
American Journal of Human Genetics, 91, 4, pp. 636-45
The American journal of human genetics

Item does not contain fulltext CaBPs are a family of Ca(2+)-binding proteins related to calmodulin and are localized in the brain and sensory organs, including the retina and cochlea. Although their physiological roles are not yet fully elucidated, C

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::44010e00ec7c130542c3cfd5a9cd721c

Nanobiotechnology advanced antifouling surfaces for the continuous electrochemical monitoring of glucose in whole blood using a lab-on-a-chip

Autor: Uwe B. Sleytr, Jakub Dostalek, Peter Ertl, Seta Küpcü, Dietmar Pum, Maria Magdalena Picher, Chun Jen Huang

Publikováno v: Lab on a chip. 13(9)

In the current work we have developed a lab-on-a-chip containing embedded amperometric sensors in four microreactors that can be addressed individually and that are coated with crystalline surface protein monolayers to provide a continuous, stable, r

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9fbce18943c0d3bd5fabcd48805bcd71
https://pubmed.ncbi.nlm.nih.gov/23478879