Results 41 to 50 of about 1,816 (142)

The peroxisomal exportomer directly inhibits phosphoactivation of the pexophagy receptor Atg36 to suppress pexophagy in yeast

eLife, 2022
Autophagy receptor (or adaptor) proteins facilitate lysosomal destruction of various organelles in response to cellular stress, including nutrient deprivation. To what extent membrane-resident autophagy receptors also respond to organelle-restricted cues
Houqing Yu, Roarke A Kamber, Vladimir Denic   +2 more
doaj   +1 more source

Recovery of PEX1-Gly843Asp peroxisome dysfunction by small-molecule compounds [PDF]

Proceedings of the National Academy of Sciences, 2010
Zellweger spectrum disorder (ZSD) is a heterogeneous group of diseases with high morbidity and mortality caused by failure to assemble normal peroxisomes. There is no therapy for ZSD, but management is supportive. Nevertheless, one-half of the patients have a phenotype milder than classic Zellweger syndrome and exhibit a progressive disease course ...
Rui, Zhang, Li, Chen, Sarn, Jiralerspong, Ann, Snowden, Steven, Steinberg, Nancy, Braverman   +5 more
openaire   +2 more sources

Peroxins in Peroxisomal Receptor Export System Contribute to Development, Stress Response, and Virulence of Insect Pathogenic Fungus Beauveria bassiana

Journal of Fungi, 2022
In filamentous fungi, recycling of receptors responsible for protein targeting to peroxisomes depends on the receptor export system (RES), which consists of peroxins Pex1, Pex6, and Pex26.
Jia Hou, Haiyan Lin, Jinli Ding, Mingguang Feng, Shenghua Ying   +4 more
doaj   +1 more source

LC-MS Based Platform Simplifies Access to Metabolomics for Peroxisomal Disorders

Metabolites, 2021
Peroxisomes are central hubs for cell metabolism and their dysfunction is linked to devastating human disorders, such as peroxisomal biogenesis disorders and single peroxisomal enzyme/protein deficiencies.
Henry Gerd Klemp, Matthias Kettwig, Frank Streit, Jutta Gärtner, Hendrik Rosewich, Ralph Krätzner   +5 more
doaj   +1 more source

Transcription factor PEX1 modulates extracellular matrix turnover through regulation of MMP-9 expression [PDF]

, 2017
The phenylephrine-induced complex-1 (PEX1) transcription factor, also known as zinc-finger protein 260 (Zfp260), is an effector of endothelin-1 and alpha(1)-adrenergic signaling in cardiac hypertrophy.
Acosta, Alicia Jurado, Moilanen, Anne-Mari, Szabo, Zoltan, Komati, Hiba, Nemer, Mona, Ruskoaho, Heikki, Rysä, Jaana   +6 more
core   +1 more source

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

Genome-Wide Methylation Profiling in the Thalamus of Scrapie Sheep

Frontiers in Veterinary Science, 2022
Scrapie is a neurodegenerative disorder belonging to the group of transmissible spongiform encephalopathy (TSE). Scrapie occurs in sheep and goats, which are considered good natural animal models of these TSE.
Adelaida Hernaiz, Arianne Sanz, Sara Sentre, Beatriz Ranera, Oscar Lopez-Pérez, Oscar Lopez-Pérez, Pilar Zaragoza, Juan J. Badiola, Hicham Filali, Rosa Bolea, Janne M. Toivonen, Janne M. Toivonen, Inmaculada Martín-Burriel, Inmaculada Martín-Burriel, Inmaculada Martín-Burriel   +14 more
doaj   +1 more source

The N1 domain of the peroxisomal AAA-ATPase Pex6 is required for Pex15 binding and proper assembly with Pex1. [PDF]

J Biol Chem
The heterohexameric ATPases associated with diverse cellular activities (AAA)-ATPase Pex1/Pex6 is essential for the formation and maintenance of peroxisomes.
Ali BA, Judy RM, Chowdhury S, Jacobsen NK, Castanzo DT, Carr KL, Richardson CD, Lander GC, Martin A, Gardner BM.   +9 more
europepmc   +2 more sources

Expanding the clinical and genetic spectrum of Heimler syndrome

Orphanet Journal of Rare Diseases, 2019
Background Heimler syndrome (HS) is a rare hereditary systemic disorder, partial clinically overlapping with Usher syndrome. So far, our knowledge of HS is very limited, many cases are misdiagnosed or may not even be diagnosed at all. This study aimed to
Feng-Juan Gao, Fang-Yuan Hu, Ping Xu, Yu-He Qi, Jian-Kang Li, Yong-Jin Zhang, Fang Chen, Qing Chang, Fang Song, Si-Mai Shen, Ge-Zhi Xu, Ji-Hong Wu   +11 more
doaj   +1 more source

A novel mutation in the PEX26 gene in a family from Dagestan with members affected by Zellweger spectrum disorder

Molecular Genetics and Metabolism Reports, 2021
Background: Peroxisome biogenesis disorders (PBD) are a heterogeneous group of autosomal recessive disorders that affect multiple organ systems. Approximately 80% of PBD patients are classifiedin the Zellweger syndrome spectrum, which is generally caused
Natalia A. Semenova, Marina V. Kurkina, Andrey V. Marakhonov, Elena L. Dadali, Natalia N. Taran, Tatyana V. Strokova   +5 more
doaj   +1 more source