Results 41 to 50 of about 1,135,071 (192)

Lipid droplet profiling during neutrophil differentiation by stimulated Raman scattering microscopy

Biotechnology Progress, EarlyView.
Abstract Lipid droplets (LDs) are dynamic organelles that serve as metabolic hubs and emerging regulators of immune cell fate. Although LDs have been implicated in immune regulation, how LD metabolism is remodeled during neutrophil differentiation and how stage‐specific LD dynamics shape mature neutrophil function remain poorly defined.
Ting‐Jung Sung, Bin Dong, Jingqiao Shen, Yan Tan, Chi Zhang, Xiaoping Bao   +5 more
wiley   +1 more source

Human disorders of peroxisome metabolism and biogenesis

Biochimica et Biophysica Acta (BBA) - Molecular Cell Research, 2016
Peroxisomes are dynamic organelles that play an essential role in a variety of cellular catabolic and anabolic metabolic pathways, including fatty acid alpha- and beta-oxidation, and plasmalogen and bile acid synthesis. Defects in genes encoding peroxisomal proteins can result in a large variety of peroxisomal disorders either affecting specific ...
Waterham, Hans R., Ferdinandusse, Sacha, Wanders, Ronald J. A.   +2 more
openaire   +3 more sources

Isolation and characterization of Pas2p, a peroxisomal membrane protein essential for peroxisome biogenesis in the methylotrophic yeast Pichia pastoris [PDF]

, 1996
The pas2 mutant of the methylotrophic yeast Pichia pastoris is characterized by a deficiency in peroxisome biogenesis. We have cloned the PpPAS2 gene by functional complementation and show that it encodes a protein of 455 amino acids with a molecular ...
Wenzel, Thibaut, Wiemer, Erik A.C., Faber, Klaas Nico, Subramani, Suresh, Veenhuis, Marten, Lüers, Georg H.   +5 more
core   +8 more sources

Inherited metabolic epilepsies–established diseases, new approaches

Epilepsia Open, EarlyView.
Abstract Inherited metabolic epilepsies (IMEs) represent the inherited metabolic disorders (IMDs) in which epilepsy is a prevailing component, often determining other neurodevelopmental outcomes associated with the disorder. The different metabolic pathways affected by individual IMEs are the basis of their rarity and heterogeneity.
Itay Tokatly Latzer, Phillip L. Pearl
wiley   +1 more source

Peroxisome biogenesis and human peroxisome-deficiency disorders

Proceedings of the Japan Academy, Series B, 2016
Peroxisome is a single-membrane-bounded ubiquitous organelle containing a hundred different enzymes that catalyze various metabolic pathways such as β-oxidation of very long-chain fatty acids and synthesis of plasmalogens. To investigate peroxisome biogenesis and human peroxisome biogenesis disorders (PBDs) including Zellweger syndrome, more than a ...
openaire   +3 more sources

Peroxisomal dysfunction interferes with odontogenesis and leads to developmentally delayed teeth and defects in distinct dental cells in Pex11b-deficient mice.

PLoS ONE
Human peroxisomal biogenesis disorders of the Zellweger syndrome spectrum affect skeletal development and induce tooth malformations. Whereas several peroxisomal knockout mouse studies elucidated the pathogenesis of skeletal defects, little information ...
Claudia Colasante, Julia Jednakowski, Klaus-Peter Valerius, Xiaoling Li, Eveline Baumgart-Vogt   +4 more
doaj   +1 more source

A founder mutation in the PEX6 gene is responsible for increased incidence of Zellweger syndrome in a French Canadian population

BMC Medical Genetics, 2012
Background Zellweger syndrome (ZS) is a peroxisome biogenesis disorder due to mutations in any one of 13 PEX genes. Increased incidence of ZS has been suspected in French-Canadians of the Saguenay-Lac-St-Jean region (SLSJ) of Quebec, but this remains ...
Levesque Sebastien, Morin Charles, Guay Simon-Pierre, Villeneuve Josee, Marquis Pascale, Yik Wing, Jiralerspong Sarn, Bouchard Luigi, Steinberg Steven, Hacia Joseph G, Dewar Ken, Braverman Nancy E   +11 more
doaj   +1 more source

Therapies for Cardiovascular‐Kidney‐Liver‐Metabolic Syndrome: Reappraisal of Fibrates

Diabetes, Obesity and Metabolism, EarlyView.
ABSTRACT Cardiovascular disease, chronic kidney disease (CKD), type 2 diabetes mellitus (T2DM), obesity and metabolic dysfunction‐associated steatohepatitis (MASH) frequently coexist and share overlapping pathophysiology, forming the proposed cardiovascular‐kidney‐liver‐metabolic (CKLM) syndrome.
Virginia Anagnostopoulou, Christoforos K. Travlos, Lucas Lage Marinho, Thomas A. Mavrakanas, João Pedro Ferreira, Abhinav Sharma   +5 more
wiley   +1 more source

Peroxisomal Disorders

, 2017
Disorders of the peroxisome are divided into two major categories. In the first, the organelle fails to develop normally, leading to disruption of multiple peroxisomal enzymes.
Mohamed Y. Jefri, Kristin W. Baranano, Gerald V. Raymond, Ali Fatemi   +3 more
core   +1 more source

The Role of Oxidative Stress in Periodontitis

Journal of Periodontal Research, EarlyView.
Oxidative stress is involved in multiple chemical reactions that take place in different intracellular organelles: mitochondria, rough endoplasmic reticulum, peroxisomes, autophagy, and aging, and can be influenced by exogenous factors: nutrition, physical activity, psychological status, environmental conditions, microbiome, and drugs.
Pedro Bullon, Francesca Giampieri, Beatriz Bullon, Maurizio Battino   +3 more
wiley   +1 more source