Results 111 to 120 of about 246,925 (304)

Peroxisomal disorders: The single peroxisomal enzyme deficiencies

open access: yesBiochimica et Biophysica Acta (BBA) - Molecular Cell Research, 2006
Peroxisomal disorders are a group of inherited diseases in man in which either peroxisome biogenesis or one or more peroxisomal functions are impaired. The peroxisomal disorders identified to date are usually classified in two groups including: (1) the disorders of peroxisome biogenesis, and (2) the single peroxisomal enzyme deficiencies.
Wanders, Ronald J.A., Waterham, Hans R.
openaire   +2 more sources

Unveiling a New Link: Cholesterol Deficiency in Smith–Lemli–Opitz and Niemann–Pick C as a Driver of Ciliopathies

open access: yesAmerican Journal of Medical Genetics Part A, EarlyView.
ABSTRACT The ciliopathies are a group of genetic disorders caused by defective function of either the primary cilia (a large number) or the motile cilia (a much smaller number). These have been defined as diseases with mutations in genes encoding individual ciliary or cilia‐associated proteins.
Robert P. Erickson   +1 more
wiley   +1 more source

A Genetic Screen for Mutants with Supersized Lipid Droplets in Caenorhabditis elegans

open access: yesG3: Genes, Genomes, Genetics, 2016
To identify genes that regulate the dynamics of lipid droplet (LD) size, we have used the genetically tractable model organism Caenorhabditis elegans, whose wild-type LD population displays a steady state of size with an upper limit of 3 μm in diameter ...
Shiwei Li   +12 more
doaj   +1 more source

Alkane-grown Beauveria bassiana produce mycelial pellets displaying peroxisome proliferation, oxidative stress, and cell surface alterations [PDF]

open access: yes, 2018
The entomopathogenic fungus Beauveria bassiana is able to grow on insect cuticle18 hydrocarbons as the sole carbon source, inducing several enzymes involved in alkane19 assimilation and concomitantly increasing virulence against insect hosts.
Huarte Bonnet, Carla   +5 more
core   +1 more source

Tree shrew model of early diabetic retinopathy reveals microvascular dysfunction and identifies phosphoserine aminotransferase 1 as a novel therapeutic target

open access: yesAnimal Models and Experimental Medicine, EarlyView.
We developed an animal model of early diabetic retinopathy (DR) in the tree shrew by feeding a high‐fat and high‐sugar diet in combination with STZ. Physiological and biochemical index detection, hematoxylin and eosin staining, and transmission electron microscopy were performed to examine DR.
Min Qiu   +10 more
wiley   +1 more source

Subcellular Energetics and Carbon Storage in Chlamydomonas

open access: yesCells, 2019
Microalgae have emerged as a promising platform for production of carbon- and energy- rich molecules, notably starch and oil. Establishing an economically viable algal biotechnology sector requires a holistic understanding of algal photosynthesis ...
Adrien Burlacot   +2 more
doaj   +1 more source

An ultra‐processed food‐based cafeteria diet induces obesity, metabolic dysfunction, and tissue‐specific gene dysregulation in C57BL/6 mice

open access: yesAnimal Models and Experimental Medicine, EarlyView.
This schematic figure illustrates the experimental design and key findings of the study. Forty male C57BL/6 mice were assigned to either a standard diet control group or a cafeteria diet (CAFD) group for 16 weeks. CAFD induces obesity, insulin resistance (HOMA‐IR), hepatic steatosis, and dysregulation of a number of genes in visceral and subcutaneous ...
Guilherme Coutinho Kullmann Duarte   +9 more
wiley   +1 more source

HDAC inhibitor SAHA normalizes the levels of VLCFAs in human skin fibroblasts from X-ALD patients and downregulates the expression of proinflammatory cytokines in Abcd1/2-silenced mouse astrocytes

open access: yesJournal of Lipid Research, 2011
X-adrenoleukodystrophy (X-ALD) is a peroxisomal metabolic disorder caused by mutations in the ABCD1 gene encoding the peroxisomal ABC transporter adrenoleukodystrophy protein (ALDP).
Jaspreet Singh   +2 more
doaj   +1 more source

Peroxisomes and peroxisomal disorders: The main facts [PDF]

open access: yesExperimental and Toxicologic Pathology, 2010
The importance of peroxisomes for human health is highlighted by the number of peroxisomal disorders (PDs), diseases associated to peroxisome biogenesis disorders and peroxisomal enzyme/transporter deficiencies. Currently, many physiological/biosynthetic mechanisms involved in these illnesses have been elucidated, but PDs remain incurable.
openaire   +3 more sources

Ciglitazone-a human PPARγ agonist-disrupts dorsoventral patterning in zebrafish. [PDF]

open access: yes, 2019
Peroxisome proliferator-activated receptor γ (PPARγ) is a ligand-activated transcription factor that regulates lipid/glucose homeostasis and adipocyte differentiation.
Cheng, Vanessa   +3 more
core  

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