Results 51 to 60 of about 4,693,382 (193)

Epoxide hydrolase in human and rat peroxisomes: implication for disorders of peroxisomal biogenesis.

Journal of Lipid Research, 1996
To understand the basis of excretion of excessive amounts of epoxydicarboxylic fatty acids (EDFA) in urine of patients with disorders of peroxisomal biogenesis (Pitt, J. J., and A. Poulos. 1993. Clin. Chim. Acta.
K Pahan, B T Smith, I Singh
doaj   +1 more source

Peroxisomal biogenesis disorders in Zellweger syndrome spectrum: diagnosis, monitoring and treatment according to the recommendations of the Global Foundation for Peroxisomal Disorders

Zdorovʹe Rebenka, 2018
The article deals with the modern principles for the diagnosis and treatment of peroxisomal biogenesis disorders of Zellweger syndrome spectrum according to the recommendations of the Global Foundation for Peroxisomal Disorders 2016.
M.A. Gonchar, G.R. Muratov, O.L. Logvinova, E.M. Pushkar, E.P. Pomazunovskaya, E.V. Omelchenko, N.V. Shulga, I.M. Galdina, A.S. Zubko, N.V. Strebul   +9 more
doaj   +1 more source

Depletion of LONP2 unmasks differential requirements for peroxisomal function between cell types and in cholesterol metabolism

Biology Direct, 2023
Peroxisomes play a central role in tuning metabolic and signaling programs in a tissue- and cell-type-specific manner. However, the mechanisms by which the status of peroxisomes is communicated and integrated into cellular signaling pathways are not yet ...
Akihiro Yamashita, Olesia Ignatenko, Mai Nguyen, Raphaëlle Lambert, Kathleen Watt, Caroline Daneault, Isabelle Robillard-Frayne, Ivan Topisirovic, Christine Des Rosiers, Heidi M. McBride   +9 more
doaj   +1 more source

Peroxisomal biogenesis is genetically and biochemically linked to carbohydrate metabolism in Drosophila and mouse.

PLoS Genetics, 2017
Peroxisome biogenesis disorders (PBD) are a group of multi-system human diseases due to mutations in the PEX genes that are responsible for peroxisome assembly and function.
Michael F Wangler, Yu-Hsin Chao, Vafa Bayat, Nikolaos Giagtzoglou, Abhijit Babaji Shinde, Nagireddy Putluri, Cristian Coarfa, Taraka Donti, Brett H Graham, Joseph E Faust, James A McNew, Ann Moser, Marco Sardiello, Myriam Baes, Hugo J Bellen   +14 more
doaj   +1 more source

Bioengineered Exosome‐Loaded Immunomodulatory Bioadhesive Spray Reverses Liver Fibrosis and Metabolic Dysfunction That Triggers Beneficial Gut‐Liver Crosstalk in Chronic Fatty Liver Disease

Advanced Science, EarlyView.
Umbilical cord stem cell‐derived exosomes encapsulated in an immunomodulatory bioadhesive hydrogel spray enable localized therapy, sustained vesicle delivery, macrophage polarization, suppress systemic inflammation, and mitigate glucose and insulin homeostasis impairments in MAFLD.
Triya Saha, Ayushi Mairal, Shreya Mehrotra, Shiv K. Sarin, Ashok Kumar   +4 more
wiley   +1 more source

Pristanic acid and phytanic acid in plasma from patients with peroxisomal disorders: stable isotope dilution analysis with electron capture negative ion mass fragmentography.

Journal of Lipid Research, 1992
A sensitive and selective stable isotope dilution method was developed for the accurate quantitation of pristanic acid and phytanic acid using electron capture negative ion mass fragmentography on pentafluorobenzyl derivatives.
HJ ten Brink, F Stellaard, CM van den Heuvel, RM Kok, DS Schor, RJ Wanders, C Jakobs   +6 more
doaj   +1 more source

Miro1 Protects Against Acute Kidney Injury Through Modulating Mitochondrial Homeostasis via Interaction With Sirt6

Advanced Science, EarlyView.
Ischemia‐reperfusion reduces Sirt6 activity, thereby increasing Miro1 acetylation. Hyperacetylated Miro1 exhibits perinuclear distribution and degradation, thereby inducing mitochondrial dysfunction and promoting apoptosis in renal tubular epithelial cells. This pathway reveals a mechanistic link between Sirt6‐mediated deacetylation and Miro1 stability
Lin Wu, Qing Li, Yiyang Xu, Zitao Wang, Hua Shu, Yangfan Wu, Fang Lu, Zhimin Huang, Bo Zhang, Hongwei Liang, Changying Xing, Huijuan Mao, Yanggang Yuan   +12 more
wiley   +1 more source

Molecular Bases and Genetic Design of Rice Disease Resistance for Optimized Yield and Sustainable Agriculture

Advanced Science, EarlyView.
This study integrates rice immune perception, signal transduction, and resistance output into a multi‐omics framework. By combining high‐throughput sequencing, phenomics, and AI‐assisted design with marker‐assisted selection, gene editing, and rapid domestication, we aim to accelerate the precise breeding of disease‐resistant rice varieties by ...
Xinyue Hou, Yuanjiang Cui, Chaoqing Ding, Qian Qian, Deyong Ren   +4 more
wiley   +1 more source

Lipoprotein[a] is not present in the plasma of patients with some peroxisomal disorders

Journal of Lipid Research, 1997
Peroxisomal disorders arise either from defects in the biogenesis of peroxisomes or from the defective synthesis of one or more peroxisomal enzymes. These defects result in metabolic disturbances in peroxisomal beta-oxidation of various fatty acids and ...
Y Y van der Hoek, R J Wanders, A E van den Ende, H G Kraft, B R Gabel, J J Kastelein, M L Koschinsky   +6 more
doaj   +1 more source

Novel DNM1L variants impair mitochondrial dynamics through divergent mechanisms

Life Science Alliance, 2022
Novel DNM1L variants underlie a spectrum of clinical phenotypes and impair mitochondrial and peroxisomal dynamics via divergent mechanisms, with effects on DRP1 protein stability, GTPase activity, and oligomerisation in vitro. Imbalances in mitochondrial
Kelsey A Nolden, John M Egner, Jack J Collier, Oliver M Russell, Charlotte L Alston, Megan C Harwig, Michael E Widlansky, Souphatta Sasorith, Inês A Barbosa, Andrew GL Douglas, Julia Baptista, Mark Walker, Deirdre E Donnelly, Andrew A Morris, Hui Jeen Tan, Manju A Kurian, Kathleen Gorman, Santosh Mordekar, Charu Deshpande, Rajib Samanta, Robert McFarland, R Blake Hill, Robert W Taylor, Monika Oláhová   +23 more
doaj   +1 more source