Results 51 to 60 of about 2,517,871 (188)

Phytanoyl-CoA hydroxylase from rat liver: protein purification and cDNA cloning with implications for the subcellular localization of phytanic acid α-oxidation

Journal of Lipid Research, 1999
Phytanoyl-CoA hydroxylase (PhyH) catalyzes the conversion of phytanoyl-CoA to 2-hydroxyphytanoyl-CoA, which is the first step in the phytanic acid α-oxidation pathway.
Gerbert A. Jansen, Rob Ofman, Simone Denis, Sacha Ferdinandusse, Eveline M. Hogenhout, Cornelis Jakobs, Ronald J.A. Wanders   +6 more
doaj   +1 more source

Diagnosis and Metabolic Management of Adult Refsum Disease: Guidance From the Medical and Scientific Committee of Global DARE (Defeat Adult Refsum Everywhere)

Journal of Inherited Metabolic Disease, Volume 49, Issue 3, May 2026.
ABSTRACT Adult Refsum disease (ARD; OMIM 266510) is a degenerative autosomal recessive condition typically diagnosed in adulthood. It affects visual, auditory and nervous system function. It is characterised by plasma, neuro‐ophthalmological and adipose tissue accumulation of the dietary‐derived phytanic acid (PA).
Radha Ramachandran, Raphael Buttigieg, Florian Eichler, Bart P. Leroy, Omar Mahroo, Rachel M. Huckfeldt, Sarah Firman, Eleanor Baldwin, Kristie DeMarco, Susan Kuranoff, Amena Fine, Reinhard Klingel, Sacha Ferdinandusse, Joseph G. Hacia, Ronald J. Wanders, Anthony S. Wierzbicki   +15 more
wiley   +1 more source

Stereochemistry of the peroxisomal branched-chain fatty acid α- and β-oxidation systems in patients suffering from different peroxisomal disorders

Journal of Lipid Research, 2002
Phytanic acid (3,7,11,15-tetramethylhexadecanoic acid) is a branched-chain fatty acid derived from dietary sources and broken down in the peroxisome to pristanic acid (2,6,10,14-tetramethylpentadecanoic acid) via α-oxidation.
S. Ferdinandusse, H. Rusch, A.E.M. van Lint, G. Dacremont, R.J.A. Wanders, P. Vreken   +5 more
doaj   +1 more source

Examining the effect of sterol carrier protein-2 on phytanic acid toxicity [PDF]

, 1998
Digitized from print original stored in HDR. Due to the character of the original source materials and the nature of batch digitization, quality control issues may be present in this document.
Chen, Cynthia
core   +1 more source

Dihydromyricetin Regulates PANoptosis to Protect Salmonella Typhimurium‐Infected Weaned Piglets

Food Frontiers, Volume 7, Issue 2, March 2026.
After DHM pretreatment, piglets were subjected to Salmonella infection. Serum and ileocecal samples from the piglets were collected for TUNEL assay, Western blotting analysis, and metabolomics analysis. The results collectively indicated that DHM could protect piglets from Salmonella infection.
Tingting Xiao, Xinyi Tian, YiKun Li, Jie Zhang, Hongcan Huang, Yu‐Xuan Lyu, Baichuan Deng   +6 more
wiley   +1 more source

Studies on the oxidation of phytanic acid and pristanic acid in human fibroblasts by acylcarnitine analysis

, 1998
The alpha-oxidation of phytanic acid and the beta-oxidation of pristanitc acid were investigated in cultured fibroblasts from controls and patients affected with different peroxisomal disorders using deuterated substrates.
Verhoeven, N. M., Jakobs, C., ten Brink, H. J., Wanders, R. J., Roe, C. R.   +4 more
core   +1 more source

Refsum's Disease—Use of the Intestinal Lipase Inhibitor, Orlistat, as a Novel Therapeutic Approach to a Complex Disorder

Journal of Obesity, 2011
Refsum's Disease is an inherited metabolic disorder in which a metabolite of branched chain fatty acids accumulates due to lack of appropriate oxidative enzymes.
Nimalie J. Perera, Barry Lewis, Huy Tran, Michael Fietz, David R. Sullivan   +4 more
doaj   +1 more source

Crosstalk between Peroxisomal Activities and Nrf2 Signaling in Porcine Embryos

Antioxidants, 2021
Melatonin and phytanic acid (PA) are known to be involved in lipid metabolism and β-oxidation, in which peroxisomal activities also significantly participate.
Eui-Hyun Kim, Muhammad-Rosyid Ridlo, Byeong-Chun Lee, Geon A. Kim   +3 more
doaj   +1 more source

Cultured Meat: A Multidimensional Review of Technological, Nutritional, Ethical, and Regulatory Advances (2020–2025)

Journal of Food Science, Volume 91, Issue 2, February 2026.
ABSTRACT Global food systems face increasing environmental, ethical, and health‐related challenges, prompting the search for sustainable protein alternatives. Cultured meat has emerged as a promising option, offering potential benefits such as reduced environmental impact, improved animal welfare, and nutritional customization.
Ana Carolina Agne Ferreira Zão, Wesclen Vilar Nogueira, Filipe Soares Rondan, Priscila Tessmer Scaglioni   +3 more
wiley   +1 more source

Biomedical Activities of Florasol

Journal of Preventive, Diagnostic and Treatment Strategies in Medicine, 2022
Florasol is an acyclic alcohol of diterpene regularly available in certain aromatic plants' essential oils, such as Lantana radula and Cleome serrata. Florasol is a plant phytochemical phytoconstituent which is extensively spread in nature. Florasol is a
Paramasivam Selvakumar
doaj   +1 more source