Results 131 to 140 of about 1,816 (142)

Peroxisomal monoubiquitinated PEX5 interacts with the AAA ATPases PEX1 and PEX6 and is unfolded during its dislocation into the cytosol [PDF]

Journal of Biological Chemistry, 2018
PEX1 and PEX6 are two members of the ATPases associated with diverse cellular activities (AAA) family and the core components of the receptor export module of the peroxisomal matrix protein import machinery.
Azevedo Je, Ana G Pedrosa, Ana Filipa Dias   +2 more
exaly   +2 more sources

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Mutations in PEX1 are the most common cause of peroxisome biogenesis disorders

Nature Genetics, 1997
The peroxisome biogenesis disorders (PBDs) are a group of lethal autosomal-recessive diseases caused by defects in peroxisomal matrix protein import, with the concomitant loss of multiple peroxisomal enzyme activities. Ten complementation groups (CGs) have been identified for the PBDs, with CG1 accounting for 51% of all PBD patients.
B E, Reuber, E, Germain-Lee, C S, Collins, J C, Morrell, R, Ameritunga, H W, Moser, D, Valle, S J, Gould   +7 more
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PEX1 is a mediator of α1‐adrenergic signaling attenuating doxorubicin‐induced cardiotoxicity

Journal of Biochemical and Molecular Toxicology, 2022
AbstractDoxorubicin (DOX) is a potent chemotherapeutic agent used for cancer treatment, however, DOX‐induced cardiotoxicity is a serious clinical problem because it causes acute and chronic heart dysfunction. Many studies have indicated that the α1‐adrenergic receptor protects the heart from pathologic stress through activation survival signaling ...
Wenjuan Li, Huilin Xie, Huang Hu, Jihong Huang, Sun Chen   +4 more
openaire   +2 more sources

PEX1 is essential for the glycosome biogenesis and trypanosomatid parasite survival

2023
Abstract Trypanosomatid parasites are kinetoplastid protists that compartmentalize glycolytic enzymes in unique peroxisome-related organelles called glycosomes. The heterohexameric AAA-ATPase complex of PEX1-PEX6 is anchored to the peroxisomal membrane and functions in the export of matrix protein import receptor PEX5
Lavanya Mahadevan, Hemant Arya, Wolfgang Schliebs, Ralf Erdmann, Vishal C. Kalel   +4 more
openaire   +1 more source

Human PEX1 is mutated in complementation group 1 of the peroxisome biogenesis disorders

Nature Genetics, 1997
Human peroxisome biogenesis disorders (PBDs) are a group of genetically heterogeneous autosomal-recessive disease caused by mutations in PEX genes that encode peroxins, proteins required for peroxisome biogenesis. These lethal diseases include Zellweger syndrome (ZS), neonatal adrenoleukodystrophy (NALD) and infantile Refsum's disease (IRD), three ...
H, Portsteffen, A, Beyer, E, Becker, C, Epplen, A, Pawlak, W H, Kunau, G, Dodt   +6 more
openaire   +2 more sources

A PEX1 terminal deletion retains partial PEX1 protein function resulting in an attenuated Zellweger spectrum phenotype

Molecular Genetics and Metabolism, 2021
Erminia Di Pietro, Catherine Argyriou, Panteha Saberian, Ellen Crushell, Steven Steinberg, Yasmin D’Souza, Nancy Braverman   +6 more
openaire   +1 more source

Ophthalmic Manifestations of Heimler Syndrome in Two Siblings With PEX1 Variants

Journal of Pediatric Ophthalmology & Strabismus
Purpose: To report two new cases with confirmed diagnosis of Heimler syndrome and describe their systemic and ophthalmic phenotype and visual rehabilitation. Methods: Retrospective review of medical records.
Vítor, Miranda, Liliana, Cortez, Joana, Rosmaninho-Salgado, Fabiana, Ramos, Catarina, Paiva   +4 more
openaire   +2 more sources

Comparison of human PEX knockout cell lines suggests a dual role of PEX1 in peroxisome biogenesis

Biological Chemistry, 2023
Ralf Erdmann, Wolfgang Schliebs, Nadine Schmidt   +2 more
exaly  

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

Cells, 2021
Etienne Sokal, Tanguy Demaret, Martin Roumain   +2 more
exaly  

Unique double-ring structure of the peroxisomal Pex1/Pex6 ATPase complex revealed by cryo-electron microscopy

Proceedings of the National Academy of Sciences of the United States of America, 2015
Ray Wang, David Baker, Tom Rapoport
exaly