Journal of Lipid Research, 1997 The degradation of the first intermediate in the alpha-oxidation of phytanic acid, 2-hydroxyphytanoyl-CoA, was investigated. Human liver homogenates were incubated with 2-hydroxyphytanoyl-CoA or 2-hydroxyphytanic acid, after which formation of 2 ...
N M Verhoeven +4 more
doaj +1 more source
Disorders of fatty acid homeostasis
Journal of Inherited Metabolic Disease, Volume 48, Issue 1, January 2025.Abstract Humans derive fatty acids (FA) from exogenous dietary sources and/or endogenous synthesis from acetyl‐CoA, although some FA are solely derived from exogenous sources (“essential FA”). Once inside cells, FA may undergo a wide variety of different modifications, which include their activation to their corresponding CoA ester, the introduction of
Frédéric M. Vaz +3 more
wiley +1 more source
Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage [PDF]
, 2017 ATP-binding cassette (ABC) transporters facilitate unidirectional translocation of chemically diverse substances, ranging from peptides to lipids, across cell or organelle membranes.
Broccoli, Vania +12 more
core
The molecular basis of human retinal and vitreoretinal diseases [PDF]
, 2010 During the last two to three decades, a large body of work has revealed the molecular basis of many human disorders, including retinal and vitreoretinal degenerations and dysfunctions.
Berger, W +2 more
core +1 more source
Absorption of chlorophyll phytol in normal man and in patients with Refsum's disease
Journal of Lipid Research, 1968 This study was made to determine the extent of absorption of chlorophyll phytol from the intestine of man, and the importance of chlorophyll as a source of the phytanic acid that accumulates in Refsum's disease. Uniformly 14C-labeled pheophytin a (the Mg-
James H. Baxter
doaj +1 more source
Phytanic acid-derived peroxisomal lipid metabolism in porcine oocytes
Theriogenology, 2020 Lipid metabolism plays an important role in oocyte maturation. The peroxisome is the fundamental mediator for this mechanism. In this study, we investigated the peroxisomal lipid metabolism in porcine oocytes. Phytanic acid (PA) was chosen as an activator of alpha-oxidation in peroxisomes.
Eui Hyun, Kim +7 more
openaire +3 more sources
Stereochemistry of the α-oxidation of 3-methyl-branched fatty acids in rat liver
Journal of Lipid Research, 1999 The stereochemistry of the α-oxidation of 3-methyl-branched fatty acids was studied in rat liver. R- and S-3-methylhexadecanoic acid were equally well α-oxidized in intact hepatocytes and homogenates.
Kathleen Croes +4 more
doaj +1 more source
Phytanic Acid Intake and Lifestyle Modifications on Quality of Life in Individuals with Adult Refsum Disease: A Retrospective Survey Analysis. [PDF]
Nutrients, 2023 Li JJ, Kim JJ, Nausheen F.
europepmc +1 more source
Peroxisomal regulation of redox homeostasis and adipocyte metabolism [PDF]
, 2019 Peroxisomes are ubiquitous cellular organelles required for specific pathways of fatty acid oxidation and lipid synthesis, and until recently their functions in adipocytes have not been well appreciated.
Klein, Samuel +4 more
core +1 more source
Management and investigation of neonatal encephalopathy: 2017 update. [PDF]
, 2017 This review discusses an approach to determining the cause of neonatal encephalopathy, as well as current evidence on resuscitation and subsequent management of hypoxic-ischaemic encephalopathy (HIE).
Hart, AR +4 more
core +1 more source