Results 31 to 40 of about 1,161,477 (260)

Identification of a novel mutation in PEX10 in a patient with attenuated Zellweger spectrum disorder: a case report

Journal of Medical Case Reports, 2017
Background The peroxisome biogenesis disorders, which are caused by mutations in any of 13 different PEX genes, include the Zellweger spectrum disorders.
Maria Blomqvist, Karin Ahlberg, Julia Lindgren, Sacha Ferdinandusse, Jorge Asin-Cayuela   +4 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

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

A Peroxisomal Lon Protease and Peroxisome Degradation by Autophagy Play Key Roles in Vitality of Hansenula polymorpha Cells [PDF]

, 2007
In eukaryote cells various mechanisms exist that are responsible for the removal of non-functional proteins. Here we show that in the yeast Hansenula polymorpha (H.
Jourdan, Stefanie, Kiel, Jan A.K.W., Klei, Ida J. van der; id_orcid, Bener Aksam, Eda, Veenhuis, Marten, Koek, Anne, Klei, Ida J. van der   +6 more
core   +2 more sources

Regulation of skeletal muscle cell plasticity by the peroxisome proliferator-activated receptor gamma coactivator 1alpha [PDF]

, 2010
Exercise triggers a pleiotropic response in skeletal muscle, which results in a profound remodeling of this tissue. Physical activity-dependent muscle fiber plasticity is regulated by a number of distinct signaling pathways.
Handschin, C.
core   +1 more source

Characterization of Severity in Zellweger Spectrum Disorder by Clinical Findings: A Scoping Review, Meta-Analysis and Medical Chart Review

Cells, 2022
Zellweger spectrum disorder (ZSD) is a rare, debilitating genetic disorder of peroxisome biogenesis that affects multiple organ systems and presents with broad clinical heterogeneity.
Mousumi Bose, Christine Yergeau, Yasmin D’Souza, David D. Cuthbertson, Melisa J. Lopez, Alyssa K. Smolen, Nancy E. Braverman   +6 more
doaj   +1 more source

Peroxisome biogenesis in Hansenula polymorpha: different mutations in genes, essential for peroxisome biogenesis, cause different peroxisomal mutant phenotypes [PDF]

, 1992
In Hansenula polymorpha, different monogenic recessive mutations mapped in either of two previously identified genes, PER1 and PER3, produced different peroxisomal mutant phenotypes.
Titorenko, V. I., Harder, Wim, Waterham, H. R., Waterham, Hans R., Haima, Peter, Titorenko, Vladimir I.,, Titorenko, Vladimir I., Harder, Wim,, Haima, Peter,, Veenhuis, Marten,, Veenhuis, M., Veenhuis, Marten, Harder, W., Haima, P., Waterham, Hans R.,   +14 more
core   +1 more source

Aging impairs peroxisome biogenesis in human B cells. [PDF]

J Gerontol A Biol Sci Med Sci
Emerging evidence highlights the critical role of cellular metabolism in immune cell activation, development, and function. Peroxisomes, key metabolic organelles, maintain metabolic homeostasis, yet their role in immune cells remains underexplored. While
Correia J, Roy PS, Holden KG, Kohut ML, Bai H.   +4 more
europepmc   +2 more sources

Pmp27 Promotes Peroxisomal Proliferation [PDF]

, 1995
Peroxisomes perform many essential functions in eukaryotic cells. The weight of evidence indicates that these organelles divide by budding from preexisting peroxisomes. This process is not understood at the molecular level.
J M Goodman, Lark, Richard H.,, Krimkevich,, Y. I., Y I Krimkevich, J M Dyer, P A Marshall, Goodman, J. M., M Veenhuis, Krimkevich, Yelena I.,, Veenhuis, Marten,, Dyer, J. M., Veenhuis, M., Marshall, Pamela A.,, Goodman, Joel M.,, Lark, R. H., Dyer, John M.,, Marshall, P. A., R H Lark   +17 more
core   +2 more sources

High Dose Versus Low Dose Syngeneic Hepatocyte Transplantation in Pex1-G844D NMRI Mouse Model is Safe but Does Not Achieve Long Term Engraftment

Cells, 2020
Genetic alterations in PEX genes lead to peroxisome biogenesis disorder. In humans, they are associated with Zellweger spectrum disorders (ZSD). No validated treatment has been shown to modify the dismal natural history of ZSD. Liver transplantation (LT)
Tanguy Demaret, Jonathan Evraerts, Joachim Ravau, Martin Roumain, Giulio G. Muccioli, Mustapha Najimi, Etienne M. Sokal   +6 more
doaj   +1 more source