Results 91 to 100 of about 6,573 (255)

Fatty acid reductases (FAR): Insights into the biosynthesis of fatty alcohols in plants and bacteria [PDF]

, 2011
Primary fatty alcohols are present in all phyla, where they serve various unique biological functions, either in structural materials or biochemical compounds.
Doan, Thuy
core  

Differences in fatty acid composition between cerebral brain lobes in juvenile pigs after fish oil feeding

, 2008
Very long-chain n-3 PUFA from fish are suggested to play a role in the development of the brain. Fish oil feeding results in higher proportions of n-3 PUFA in the brains of newborn piglets. However, the effect of fish oil on the fatty acid composition of
Coronel, R., den Ruijter, Hester M., Brummer, Robert-Jan M., Den Ruijter, H.M., Kok, F.J., Ruijter, H.M., den, Brouwer, Ingeborg A., Brouwer, I.A.; id_orcid, Katan, Martijn B., Brummer, R.J., Kok, Frans J., Brummer, Robert,, Zock, Peter L., Brouwer, I.A., Beekman, J., Ruijter, H.M. den, Dullemeijer, C., Katan, M.B.; id_orcid, Zock, P.L., Beekman, Jet, Katan, M.B., Coronel, Ruben, Dullemeijer, Carla   +22 more
core   +1 more source

Application of a diagnostic methodology by quantification of 26:0 lysophosphatidylcholine in dried blood spots for Japanese newborn screening of X-linked adrenoleukodystrophy

Molecular Genetics and Metabolism Reports, 2017
X-linked adrenoleukodystrophy (X-ALD) is a rare inherited metabolic disease that results in the accumulation of very long chain fatty acids (VLCFA) in plasma and all tissues.
Chen Wu, Takeo Iwamoto, Junko Igarashi, Takashi Miyajima, Mohammad Arif Hossain, Hiroko Yanagisawa, Keiko Akiyama, Haruo Shintaku, Yoshikatsu Eto   +8 more
doaj   +1 more source

RNA Sequencing Resolves Cryptic Pathogenic Variants in Mitochondrial Disease

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Mitochondrial diseases are the most common inherited metabolic disorders, characterized by pronounced clinical and genetic heterogeneity that complicates molecular diagnosis. Although DNA‐based sequencing approaches have become standard in genetic testing, up to half of patients remain without a definitive diagnosis.
Zhimei Liu, Xin Duan, Fatemeh Peymani, Jia Wang, Chengjia Bao, Chaolong Xu, Ying Zou, Zixuan Zhang, Yunxi Zhang, Tongyue Li, Martin Pavlov, Junling Wang, Minhan Song, Tianyu Song, Xiaodi Han, Mingxi Sun, Danmin Shen, Ruoyu Duan, Huafang Jiang, Manting Xu, Holger Prokisch, Fang Fang   +21 more
wiley   +1 more source

Very-Long-Chain Fatty Acids Are Involved in Polar Auxin Transport and Developmental Patterning in \u3ci\u3eArabidopsis\u3c/i\u3e

, 2010
Very-long-chain fatty acids (VLCFAs) are essential for many aspects of plant development and necessary for the synthesis of seed storage triacylglycerols, epicuticular waxes, and sphingolipids. Identification of the acetyl-CoA carboxylase PASTICCINO3 and
Tellier, F., Bellec, Y., Jessica Marion, Napier, Johnathan A., Beaudoin, Frédéric, Molino, D., Roudier, Francois, Halima Morin, Christine Rochat, Richard Haslam, Liên Bach, Faure, J-D., François Roudier, Patrick Moreau, Palauqui, Jean-Christophe, DaCosta, Marco, Louise Michaelson, Marion, Jessica, Tellier, Frédérique, Martine Miquel, Haslam, R. P., Vignard, J., Frédérique Tellier, Markham, J. E., Michaelson, Louise V., Markham, Jennifer E., Faure, Jean Denis, Haslam, Richard, Markham, Jonathan E., Rochat, C., Napier, J. A., Renne, C., Palauqui, J-C., Roudier, F., Jennifer E. Markham, Lima, A., Lionel Gissot, Rochat, Christine, Napier, Jonathan, Michaelson, Louise, V., Lima, Amparo, Jean-Denis Faure, Miquel, Martine, Moreau, P., Johnathan Napier, Gissot, Lionel, Charlotte Renne, Beaudoin, F., Miquel, M., Dacosta, M., Jean-Christophe Palauqui, Morin, Halima, Bach, L., Roudier, Françoise, Renne, Charlotte, Morin, H., Michaelson, L. V., Marion, J., Amparo Lima, Vignard, Julien, Michaelson, Louise, Molino, Diana, Julien Vignard, Bach, Liên, Moreau, Patrick, Yannick Bellec, Bellec, Yannick, Faure, Jean-Denis, Diana Molino, Frédéric Beaudoin, Gissot, L., Marco DaCosta   +71 more
core   +1 more source

Elongation of long-chain fatty acids in rabbitfish Siganus canaliculatus: Cloning, functional characterisation and tissue distribution of Elovl5- and Elovl4-like elongases [PDF]

, 2012
Elongases of very long-chain fatty acids (Elovl) catalyse the rate-limiting step of the elongation pathway that results in net 2 C elongation of pre-existing fatty acyl chains.
You, Cuihong, Zhang, Liang, Monroig, Oscar, Tocher, Douglas R, Tocher, Douglas R., Wang, Shuqi, Li, Yuanyou   +6 more
core   +1 more source

Safety and tolerance of the ketogenic diet in patients with Zellweger Syndrome

Epilepsy & Behavior Reports
Zellweger Syndrome is a peroxisomal disorder that can lead to elevation of long chain fatty acids and epilepsy, which can be drug resistant. The treatment of drug resistant epilepsy can include the ketogenic diet in appropriately chosen patients ...
Borst Stephanie, Ciliberto Michael, Thati Ganganna Sreenath   +2 more
doaj   +1 more source

Peroxisomal Disorders Diagnosis

Pediatric Neurology Briefs, 1998
The clinical manifestations of 27 patients affected with peroxisomal disorders and seen between 1982 and 1997 are described from the Hopital Necker-Enfants Malades, Paris, and other centers.
J Gordon Millichap
doaj   +1 more source

Prognostic Value of Neurofilament Light Chain and Glial Fibrillary Acidic Protein in ALD‐Related Myelopathy

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Background X‐linked adrenoleukodystrophy (X‐ALD) is a neurometabolic disorder caused by pathogenic variants in ABCD1, leading to slowly progressive spinal cord disease in nearly all affected men. Sensitive biomarkers to quantify disease severity and predict progression are needed for clinical care and trial design.
Eda G. Kabak, Marije M. C. Voermans, Hans Heijst, Charlotte E. Teunissen, Marc Engelen   +4 more
wiley   +1 more source

Preeclampsia is associated with compromized maternal synthesis of long chain polyunsaturated fatty acids leading to offspring deficiency [PDF]

, 2012
Obesity and excessive lipolysis are implicated in preeclampsia (PE). Intrauterine growth restriction is associated with low maternal body mass index and decreased lipolysis.
Martin, I, Brown, E.A., Huda, S.S., Freeman, DJ, Jordan, F, Greer, I.A., Mackay, V.A., Brown, EA, Meyer, BJ, Tham, K, Meyer, B.J., Stewart, FM, McKenna, L.A., Huda, SS, Jordan, F., Mackay, VA, Hodson, L., Freeman, D.J., Tham, K., Martin, I., Greer, IA, Stewart, F.M., McKenna, LA, Hodson, Leanne, Hodson, L   +24 more
core   +1 more source