Phytanic acid and pristanic acid, branched-chain fatty acids associated with Refsum disease and other inherited peroxisomal disorders, mediate intracellular Ca2+ signaling through activation of free fatty acid receptor GPR40

2011 ◽  
Vol 43 (2) ◽  
pp. 465-472 ◽  
Author(s):  
Nicol Kruska ◽  
Georg Reiser
Keyword(s):  
Fatty Acids ◽  
Fatty Acid ◽  
Free Fatty Acid ◽  
Phytanic Acid ◽  
Refsum Disease ◽  
Pristanic Acid ◽  
Peroxisomal Disorders ◽  
Branched Chain Fatty Acids ◽  
Free Fatty Acid Receptor ◽  
Branched Chain

scholarly journals Effect of Rearing System on the Straight and Branched Fatty Acids of Goat Milk and Meat of Suckling Kids

Foods ◽  
2020 ◽  
Vol 9 (4) ◽  
pp. 471
Author(s):  
Guillermo Ripoll ◽  
María Jesús Alcalde ◽  
Anastasio Argüello ◽  
María de Guía Córdoba ◽  
Begoña Panea
Keyword(s):  
Fatty Acids ◽  
Fatty Acid ◽  
Goat Milk ◽  
Lower Percentage ◽  
Dairy Goat ◽  
Branched Chain Fatty Acids ◽  
Branched Chain ◽  
Milk Replacers ◽  
Fatty Acid Compositions ◽  
Chain Fatty Acids

Goat meat is considered healthy because it has fewer calories and fat than meat from other traditional meat species. It is also rich in branched chain fatty acids that have health advantages when consumed. We studied the effects of maternal milk and milk replacers fed to suckling kids of four breeds on the straight and branched fatty acid compositions of their muscle. In addition, the proximal and fatty acid compositions of colostrum and milk were studied. Goat colostrum had more protein and fat and less lactose than milk. Goat milk is an important source of healthy fatty acids such as C18:1 c9 and C18:2 n–6. Suckling kid meat was also an important source of C18:1c9. Dairy goat breeds had higher percentages of trans monounsaturated fatty acids (MUFAs) and most of the C18:1 isomers but lower amounts of total MUFAs than meat breeds. However, these dairy kids had meat with a lower percentage of conjugated linoleic acid (CLA) than meat kids. The meat of kids fed natural milk had higher amounts of CLA and branched chain fatty acids (BCFAs) and lower amounts of n–6 fatty acids than kids fed milk replacers. Both milk and meat are a source of linoleic, α-linolenic, docosahexaenoic, eicosapentaenoic and arachidonic fatty acids, which are essential fatty acids and healthy long-chain fatty acids.

FERMENTATION OF WHEAT OR LUPINS IN THE RUMEN OF SHEEP

1984 ◽  
Vol 64 (5) ◽  
pp. 29-30 ◽  
Author(s):  
R. W. HODGE ◽  
M. J. WATSON ◽  
C. KAT
Keyword(s):  
Fatty Acids ◽  
Fatty Acid ◽  
Key Words ◽  
Volatile Fatty Acid ◽  
Ammonia Nitrogen ◽  
Total Volatile ◽  
Volatile Fatty Acid Concentration ◽  
Branched Chain Fatty Acids ◽  
Branched Chain ◽  
Total Volatile Fatty Acid

The concentration of ammonia nitrogen and branched chain fatty acids was significantly higher (P < 0.05) in ruminal fluid of sheep given lupins than in those given wheat but there were no significant differences in pH, total volatile fatty acid concentration or molar proportions of acetate, propionate or butyrate. Key words: Sheep, wheat, lupins, rumen, fermentation

Biosynthesis of Branched-chain Fatty Acid inBacilli: FabD (malonyl-CoA:ACP transacylase) Is Not Essential forIn VitroBiosynthesis of Branched-chain Fatty Acids

2003 ◽  
Vol 67 (10) ◽  
pp. 2106-2114 ◽  
Author(s):  
Hirosuke OKU ◽  
Naoya FUTAMORI ◽  
Kenichi MASUDA ◽  
Yumiko SHIMABUKURO ◽  
Tomoyo OMINE ◽  
...  
Keyword(s):  
Fatty Acids ◽  
Fatty Acid ◽  
Chain Fatty Acid ◽  
Branched Chain Fatty Acids ◽  
Branched Chain ◽  
Chain Fatty Acids

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

2011 ◽  
Vol 2011 ◽  
pp. 1-5 ◽  
Author(s):  
Nimalie J. Perera ◽  
Barry Lewis ◽  
Huy Tran ◽  
Michael Fietz ◽  
David R. Sullivan
Keyword(s):  
Fatty Acids ◽  
Phytanic Acid ◽  
Retinal Dystrophy ◽  
Neurological Deficits ◽  
Oxidative Enzymes ◽  
Lipase Inhibitor ◽  
Branched Chain Fatty Acids ◽  
Refsum's Disease ◽  
Branched Chain ◽  
Chain Fatty Acids

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. Patients have elevated plasma phytanic acid levels and high concentrations of phytanic acid in a variety of tissues leading to progressive tissue damage. Besides retinal degeneration or retinal dystrophy associated with adult onset retinitis pigmentosa, additional symptoms include chronic polyneuropathy, cerebellar ataxia, sensorineural hearing loss, anosmia, ichthyosis, as well as skeletal, cardiac, hepatic, and renal abnormalities. Current management includes avoidance of dietary sources of branched chain fatty acids and regular plasmapheresis to prevent accumulation of these compounds to ameliorate progressive neurological deficits. Two brothers with Refsum's disease who experienced progressive symptoms despite optimal diet and plasmapheresis were commenced on a novel therapy. We report the effect of the intestinal lipase inhibitor, Orlistat, which led to significant reduction (P-value<0.001on 2-sample unpairedt-test) of mean preplasmapheresis phytanic acid levels with retardation of the progression of most of their dermatological and neurological symptoms.

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