Results 31 to 40 of about 1,353,029 (249)

Mitochondrial biogenesis in developing astrocytes regulates astrocyte maturation and synapse formation

Cell Reports, 2021
Summary: The mechanisms controlling the post-natal maturation of astrocytes play a crucial role in ensuring correct synaptogenesis. We show that mitochondrial biogenesis in developing astrocytes is necessary for coordinating post-natal astrocyte ...
Tamara Zehnder, Francesco Petrelli, Jennifer Romanos, Eva C. De Oliveira Figueiredo, Tommy L. Lewis, Jr., Nicole Déglon, Franck Polleux, Mirko Santello, Paola Bezzi   +8 more
doaj   +1 more source

Lipidomic analysis of fibroblasts from Zellweger spectrum disorder patients identifies disease-specific phospholipid ratios[S]

Journal of Lipid Research, 2016
Peroxisomes are subcellular organelles involved in various metabolic processes, including fatty acid and phospholipid homeostasis. The Zellweger spectrum disorders (ZSDs) represent a group of diseases caused by a defect in the biogenesis of peroxisomes ...
Katharina Herzog, Mia L. Pras-Raves, Martin A.T. Vervaart, Angela C.M. Luyf, Antoine H.C. van Kampen, Ronald J.A. Wanders, Hans R. Waterham, Frédéric M. Vaz   +7 more
doaj   +1 more source

Pay32p of the Yeast Yarrowia lipolytica Is an Intraperoxisomal Component of the Matrix Protein Translocation Machinery [PDF]

, 1995
Pay mutants of the yeast Yarrowia lipolytica fail to assemble functional peroxisomes. One mutant strain, pay32-1, has abnormally small peroxisomes that are often found in clusters surrounded by membranous material.
Rachubinski, Richard A., Szilard, Rachel K., Rachubinski, Richard A.,, Titorenko, Vladimir I.,, V I Titorenko, R A Rachubinski, Titorenko, Vladimir I., Rachel K. Szilard, M Veenhuis, Veenhuis, Marten,, R K Szilard, Szilard, Rachel K.,, Richard A. Rachubinski, Marten Veenhuis, Veenhuis, Marten, Vladimir I. Titorenko   +15 more
core   +2 more sources

Peroxisome biogenesis deficiency attenuates the BDNF-TrkB pathway-mediated development of the cerebellum

Life Science Alliance, 2018
Peroxisome biogenesis deficiency leads to increased expression of BDNF and of a truncated form of the BDNF receptor in the cerebellum, attenuates BDNF-TrkB signaling, and results in malformation of the cerebellum.
Yuichi Abe, Masanori Honsho, Ryota Itoh, Ryoko Kawaguchi, Masashi Fujitani, Kazushirou Fujiwara, Masaaki Hirokane, Takashi Matsuzaki, Keiko Nakayama, Ryohei Ohgi, Toshihiro Marutani, Keiichi I Nakayama, Toshihide Yamashita, Yukio Fujiki   +13 more
doaj   +1 more source

Hansenula polymorpha: An attractive model organism for molecular studies of peroxisome biogenesis and function [PDF]

, 1992
In wild-type Hansenula polymorpha the proliferation of peroxisomes is induced by various unconventional carbon- and nitrogen sources. Highest induction levels, up to 80% of the cytoplasmic volume, are observed in cells grown in methanol-limited chemostat
Titorenko, V.,, Titorenko, V., Klei, I.J. van der; id_orcid, Klei, I.J. van der,, Veenhuis, M., Harder, W.,, Klei, I.J. van der, Veenhuis, M.,, Harder, W.   +8 more
core   +2 more sources

Genotype–phenotype correlations and disease mechanisms in PEX13-related Zellweger spectrum disorders

Orphanet Journal of Rare Diseases, 2022
Background Pathogenic variants in PEX-genes can affect peroxisome assembly and function and cause Zellweger spectrum disorders (ZSDs), characterized by variable phenotypes in terms of disease severity, age of onset and clinical presentations.
Paola Borgia, Simona Baldassari, Nicoletta Pedemonte, Ebba Alkhunaizi, Gianluca D’Onofrio, Domenico Tortora, Elisa Calì, Paolo Scudieri, Ganna Balagura, Ilaria Musante, Maria Cristina Diana, Marina Pedemonte, Maria Stella Vari, Michele Iacomino, Antonella Riva, Roberto Chimenz, Giuseppe D. Mangano, Mohammad Hasan Mohammadi, Mehran Beiraghi Toosi, Farah Ashrafzadeh, Shima Imannezhad, Ehsan Ghayoor Karimiani, Andrea Accogli, Maria Cristina Schiaffino, Mohamad Maghnie, Miguel Angel Soler, Karl Echiverri, Charles K. Abrams, Pasquale Striano, Sara Fortuna, Reza Maroofian, Henry Houlden, Federico Zara, Chiara Fiorillo, Vincenzo Salpietro   +34 more
doaj   +1 more source

Case Report: Zellweger Syndrome and Humoral Immunodeficiency: The Relevance of Newborn Screening for Primary Immunodeficiency

Frontiers in Pediatrics, 2022
BackgroundZellweger syndrome (ZS) is a congenital autosomal recessive disease within the spectrum of peroxisome biogenesis disorders, characterized by the impairment of peroxisome assembly.
C. Fazi, L. Lodi, L. Lodi, L. Magi, C. Canessa, M. Giovannini, C. Pelosi, F. Pochiero, E. Procopio, M. A. Donati, C. Azzari, C. Azzari, S. Ricci, S. Ricci   +13 more
doaj   +1 more source

Peroxisome proliferator-activated receptor γ coactivator 1 coactivators, energy homeostasis, and metabolism [PDF]

, 2006
Many biological programs are regulated at the transcriptional level. This is generally achieved by the concerted actions of several transcription factors. Recent findings have shown that, in many cases, transcriptional coactivators coordinate the overall
Christoph Handschin, Bruce M. Spiegelman, Handschin, C., Spiegelman, B. M.   +3 more
core   +2 more sources

MicroRNAs upregulated during HIV infection target peroxisome biogenesis factors: Implications for virus biology, disease mechanisms and neuropathology. [PDF]

PLoS Pathogens, 2017
HIV-associated neurocognitive disorders (HAND) represent a spectrum neurological syndrome that affects up to 25% of patients with HIV/AIDS. Multiple pathogenic mechanisms contribute to the development of HAND symptoms including chronic neuroinflammation ...
Zaikun Xu, Eugene L Asahchop, William G Branton, Benjamin B Gelman, Christopher Power, Tom C Hobman   +5 more
doaj   +1 more source

Lipidomic profiling of PEX11β knockout T-REx 293 cells reveals lipid shifts suggesting potential metabolic perturbations

Biochemistry and Biophysics Reports
Peroxisomes function in reactive oxygen species (ROS) and lipid metabolism. Genetic defects in peroxisome biogenesis cause severe neurological disorders collectively termed peroxisome biogenesis disorders (PBDs).
Samantha Jeng, Mansha Soni, Vishal C. Kalel, Wolfgang Schliebs, Ralf Erdmann, Frank Schulz   +5 more
doaj   +1 more source