Výsledky vyhledávání - "metabolism [HSP40 Heat-Shock Proteins]"

Knocking out C9ORF72 Exacerbates Axonal Trafficking Defects Associated with Hexanucleotide Repeat Expansion and Reduces Levels of Heat Shock Proteins

Autor: Florian Wegner, Selma Staege, Andreas Hermann, Stefan Stefanov, Hannes Glass, Susanne Petri, Dieter Edbauer, Rajat Bhatnagar, Antje Janosch, Petra Freitag, Anne-Karin Kahlert, Marc Bickle, Jared Sterneckert, Tanja Richter, Arun Pal, Carina Schludi, Walter Just, Norman Kalmbach, Masin Abo-Rady

Publikováno v: Stem Cell Reports, Vol 14, Iss 3, Pp 390-405 (2020)
Stem Cell Reports
Stem cell reports 14(3), 390-405 (2020). doi:10.1016/j.stemcr.2020.01.010

Summary In amyotrophic lateral sclerosis (ALS) motor neurons (MNs) undergo dying-back, where the distal axon degenerates before the soma. The hexanucleotide repeat expansion (HRE) in C9ORF72 is the most common genetic cause of ALS, but the mechanism

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3e36d802ed14d96d26a9814e300c2698
http://www.sciencedirect.com/science/article/pii/S221367112030031X

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Impaired complex I repair causes recessive Leber’s hereditary optic neuropathy

Autor: Yulya S. Itkis, Maja Hempel, Ben Pode-Shakked, Piero Barboni, N.L. Sheremet, Polina G. Tsygankova, Riccardo Berutti, Valerio Carelli, Chiara La Morgia, Daniele Ghezzi, Leonardo Caporali, Jean-Michel Rozet, Natalia A. Andreeva, Amelie T van der Ven, Peter Charbel Issa, Wolfram S. Kunz, Sarah L. Stenton, Claudia B. Catarino, Johannes A. Mayr, Matias Wagner, Maria Lucia Cascavilla, Flavia Palombo, Reka Kovacs-Nagy, Ilka Wittig, Alessandra Maresca, Pedro Felipe Malacarne, Thomas Klopstock, Costanza Lamperti, Sylvie Gerber, Cornelia Kornblum, Holger Prokisch, Nino V. Zhorzholadze, Jana Meisterknecht, Robert Kopajtich, Tatiana A. Nevinitsyna, Ekaterina Zakharova, Michele Carbonelli, Tatiana D. Krylova, Michal Tzadok, Elisabeth Graf, Zahra Assouline, Francesca Tagliavini, Josseline Kaplan, Maria S. Shmelkova, Mariantonietta Capristo, Elise Héon, Ortal Barel, Peter Freisinger, Elisheva Javasky, Igor Bychkov, Christina Ludwig, Tim M. Strom, Catherine Vignal-Clermont, Juliana Heidler

Publikováno v: The journal of clinical investigation 131(6), e138267 (2021). doi:10.1172/JCI138267
J Clin Invest

Leber's hereditary optic neuropathy (LHON) is the most frequent mitochondrial disease and was the first to be genetically defined by a point mutation in mitochondrial DNA (mtDNA). A molecular diagnosis is achieved in up to 95% of cases, the vast majo

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::24bf545cda9fb173a8ffc9ec90e829e6
https://doi.org/10.1172/jci138267

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Cytosolic Hsp70 and Hsp40 chaperones enable the biogenesis of mitochondrial β-barrel proteins

Autor: Doron Rapaport, Viktor Beke, Mirita Franz-Wachtel, Toshiya Endo, Hubert Kalbacher, Julia C. Fitzgerald, Jannis Lawatscheck, Tobias Jores, Boris Macek, Kaori Yunoki, Johannes Buchner

Publikováno v: The Journal of Cell Biology
The journal of cell biology 217(9), 3091-3108 (2018). doi:10.1083/jcb.201712029

Mitochondrial β-barrel proteins are imported from the cytosol into the organelle. Jores et al. provide new insights into the early events of this process by describing an array of cytosolic chaperones and cochaperones that associate with newly synth

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5cff32916d7b39a6637f9032491c8287
https://doi.org/10.1083/jcb.201712029

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Mapping Interactions with the Chaperone Network Reveals Factors that Protect Against Tau Aggregation

Autor: Bryan M. Dunyak, Jungsoon Lee, Mark R. Wilson, Harindranath Kadavath, Victoria A. Assimon, Francis T.F. Tsai, Olivier Julien, Chad A. Dickey, Jennifer N. Rauch, Markus Zweckstetter, Rebecca Freilich, Sue-Ann Mok, Anne T. Gillies, Jason E. Gestwicki, Carlo Condello, Javier Oroz, Taylor Arhar

Publikováno v: Nature structural & molecular biology
Nature Structural and Molecular Biology
Nature structural & molecular biology 25(5), 384-393 (2018). doi:10.1038/s41594-018-0057-1
Nature structural & molecular biology, vol 25, iss 5

A network of molecular chaperones is known to bind proteins (“clients”) and balance their folding, function and turnover. However, it is often not clear which chaperones are critical for selective recognition of individual clients. It is also not

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3599a70c0b3cd7e1a62eb1d691ffda3f
http://europepmc.org/articles/PMC5942583

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LuTHy: a double-readout bioluminescence-based two-hybrid technology for quantitative mapping of protein-protein interactions in mammalian cells

Autor: Kirstin Rau, Sabrina Kruse, Craig C. Garner, Sabrina Golusik, Martina Zenkner, Simona Kostova, Erich E. Wanker, Sheila Hoffmann, Aline Schulz, Christopher Secker, Anne Tempelmeier, Konrad Klockmeier, Philipp Trepte, Alexander Buntru, Sigrid Schnoegl, Lisa Diez

Publikováno v: Molecular systems biology 14(7), e8071 (2018). doi:10.15252/msb.20178071
Molecular Systems Biology

Information on protein–protein interactions (PPIs) is of critical importance for studying complex biological systems and developing therapeutic strategies. Here, we present a double‐readout bioluminescence‐based two‐hybrid technology, termed

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::6ea7927e62de6c0b937b1ca46d16ec18
http://edoc.mdc-berlin.de/17591/1/17591oa.pdf

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A cross-species approach to identify transcriptional regulators exemplified for Dnajc22 and Hnf4a

Autor: Anna C. Aschenbrenner, M Brondolin, Pilar Carrera, Michael Hoch, Joachim L. Schultze, Lorenzo Bonaguro, Thomas Ulas, Kathrin Klee, Kevin Bassler

Publikováno v: Scientific Reports
Scientific Reports, Vol 7, Iss 1, Pp 1-15 (2017)
Scientific reports 7(1), 4056 (2017). doi:10.1038/s41598-017-04370-9

There is an enormous need to make better use of the ever increasing wealth of publicly available genomic information and to utilize the tremendous progress in computational approaches in the life sciences. Transcriptional regulation of protein-coding

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::7569931836e149e049272802daf6c5b2
https://pubmed.ncbi.nlm.nih.gov/28642491

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Upregulation of miR-370 and miR-543 is associated with reduced expression of heat shock protein 40 in spinocerebellar ataxia type 3

Autor: Sybille Krauß, Frank Matthes, Bernd O. Evert, Oliver Brüstle, Johannes Jungverdorben, Ashish Rajput, Michael Peitz, Stephanie Weber, Rohit Nalavade, Stefan Bonn

Publikováno v: PLoS ONE
PLoS ONE, Vol 13, Iss 8, p e0201794 (2018)
PLOS ONE 13(8), e0201794 (2018). doi:10.1371/journal.pone.0201794

Molecular chaperones are important regulators of protein folding and proteasomal removal of misfolded proteins. In spinocerebellar ataxia type 3 (SCA3), the co-chaperone DnaJ homology subfamily B member 1 (DNAJB1 or heat shock protein 40) is recruite

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d73056e927965b7cc6a8a03e0f5c25c7
https://doi.org/10.1371/journal.pone.0201794

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Absence of BiP Co-chaperone DNAJC3 Causes Diabetes Mellitus and Multisystemic Neurodegeneration

Publikováno v: The American journal of human genetics 95(6), 689-697 (2014). doi:10.1016/j.ajhg.2014.10.013
Am. J. Hum. Genet. 95, 689-697 (2014)

Diabetes mellitus and neurodegeneration are common diseases for which shared genetic factors are still only partly known. Here, we show that loss of the BiP (immunoglobulin heavy-chain binding protein) co-chaperone DNAJC3 leads to diabetes mellitus a

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d7b2de1ea656c03d41b228c328278459

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