Results 71 to 80 of about 2,517,871 (188)

Successful Treatment of Severe Hepatopulmonary Syndrome as a Rare Complication of Zellweger Spectrum Disorder

JIMD Reports, Volume 66, Issue 5, September 2025.
ABSTRACT We report the case of an 11‐year‐old girl who developed hepatopulmonary syndrome (HPS) as a rare complication of Zellweger spectrum disorder and was successfully treated with liver transplantation. Our patient presented with neonatal sensorineural hearing loss.
Riya Mary Tharakan, Sanjay Rajwal, Bernd C. Schwahn   +2 more
wiley   +1 more source

Metabolism of phytol to phytanic acid in the mouse, and the role of PPARα in its regulation

Journal of Lipid Research, 2007
Phytol, a branched-chain fatty alcohol, is the naturally occurring precursor of phytanic and pristanic acid, branched-chain fatty acids that are both ligands for the nuclear hormone receptor peroxisome proliferator-activated receptor α (PPARα).
J. Gloerich, D.M. van den Brink, J. P.N. Ruiter, N. van Vlies, F.M. Vaz, R. J.A. Wanders, S. Ferdinandusse   +6 more
doaj   +1 more source

Potential Applications of Zingiber officinale, Clitoria ternatea, and Coccinia grandis for Enhancing the Functionality of Set Yogurts

Food Science &Nutrition, Volume 13, Issue 7, July 2025.
In this study, aqueous extracts of ginger, blue butterfly pea flower, and ivy leaf were fortified to the yogurts and evaluated the sensorial, physicochemical, and functional characteristics of yogurt along with the protein interactions. Results revealed that the fortification of all the plant extracts had a significant impact on the sensorial ...
Yohan Jayaweera, Mayumi Silva, Jayani Chandrapala, Rumesh Liyanage, K. G. L. R. Jayathunge, Amali U. Alahakoon   +5 more
wiley   +1 more source

Further studies on the substrate spectrum of phytanoyl-CoA hydroxylase

Journal of Lipid Research, 2003
Refsum disease is a peroxisomal disorder characterized by adult-onset retinitis pigmentosa, anosmia, sensory neuropathy, ataxia, and an accumulation of phytanic acid in plasma and tissues.
Veerle Foulon, Stanny Asselberghs, Wendy Geens, Guy P. Mannaerts, Minne Casteels, Paul P. Van Veldhoven   +5 more
doaj   +1 more source

The effect of pathological concentrations of phytanic acid on the fatty acid composition, vitamin E concentration and function of membranes of cultured mammalian retinal cells [PDF]

, 1998
The purpose of this study was to investigate possible mechanisms for the retinal degeneration seen in adult Refsum disease. This is an inherited disorder in which phytanic acid, a dietary branched chain fatty acid, cannot be catabolised and as a result ...
Young, Sarah Phyllis
core   +1 more source

Scaling Cultured Meat: Challenges and Solutions for Affordable Mass Production

Comprehensive Reviews in Food Science and Food Safety, Volume 24, Issue 4, July 2025.
ABSTRACT As the global population grows and meat consumption increases, the demand for sustainable and efficient food systems becomes urgent. Cultured meat (CM) has emerged as a promising alternative to conventional meat, offering potential benefits in environmental conservation, resource efficiency, and animal welfare.
Huiwen Gu, Yan Kong, Dejian Huang, Youfa Wang, Vijaya Raghavan, Jin Wang   +5 more
wiley   +1 more source

Impact of Fabp1/Scp-2/Scp-x gene ablation (TKO) on hepatic phytol metabolism in mice[S]

Journal of Lipid Research, 2017
Studies in vitro have suggested that both sterol carrier protein-2/sterol carrier protein-x (Scp-2/Scp-x) and liver fatty acid binding protein [Fabp1 (L-FABP)] gene products facilitate hepatic uptake and metabolism of lipotoxic dietary phytol.
Stephen M. Storey, Huan Huang, Avery L. McIntosh, Gregory G. Martin, Ann B. Kier, Friedhelm Schroeder   +5 more
doaj   +1 more source

The Role of Metabolic Testing in the Diagnostic Evaluation of Adult NORSE: A Retrospective, Single‐Centre Study

European Journal of Neurology, Volume 32, Issue 6, June 2025.
ABSTRACT Background New‐onset refractory status epilepticus (NORSE) is a diagnostically challenging and severe epileptic presentation in which aetiology is an important predictor of outcome. This retrospective study aimed to investigate the utility of metabolic screening to determine the underlying cause in 42 patients with suspected NORSE, admitted to
Jennifer Kilmer, George Ransley, Elaine Murphy, Michael G. Hanna, Robert D. S. Pitceathly, Sanjeev Rajakulendran, Chiara Pizzamiglio   +6 more
wiley   +1 more source

Refsum's disease: a peroxisomal disorder affecting phytanic acid alpha-oxidation

, 2002
Refsum's disease (hereditary motor sensory neuropathy type IV, heredopathia atactica polyneuritiformis) is an autosomal recessive disorder the clinical features of which include retinitis pigmentosa, blindness, anosmia, deafness, sensory neuropathy ...
Gibberd, F B, Lloyd, M D, Schofield, C J, Wierzbicki, A S, Feher, M D   +4 more
core   +5 more sources

Phytanic acid alpha-oxidation: decarboxylation of 2-hydroxyphytanoyl-CoA to pristanic acid in human liver

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, R J Wanders, D S Schor, G A Jansen, C Jakobs   +4 more
doaj   +1 more source