Tapetoretinal Degenerations and Disorders of Lipid Metabolism. Part II: Biochemical Aspects

1974 ◽  
Vol 23 (S1) ◽  
pp. 33-47 ◽  
Author(s):  
E.R. Berman
Keyword(s):  
Fatty Acid ◽  
Lipid Metabolism ◽  
Phytanic Acid ◽  
Plasma Lipids ◽  
Late Onset ◽  
Neuronal Ceroid Lipofuscinosis ◽  
The Body ◽  
Plasma Lipoproteins ◽  
Acid Oxidase ◽  
Biochemical Basis

Inherited tapetoretinal degenerations associated with, or caused by, abnormalities in lipid metabolism are discussed in terms of recent findings regarding their etiology. The biochemical basis of these tapetoretinal degenerations may be summarized as follows.(a) In abetalipoproteinemia (the Bassen-Kornzweig syndrome) there is a complete absence of all plasma lipoproteins except HDL (alfalipoprotein; high density lipoprotein). In addition the levels of plasma lipids, including vitamin A, are grossly dimished. The genetically-caused basic defect in this disorder is the absence of a specific protein component, apoLP-ser, in the plasma lipoproteins.(b) In a milder form of hypobetalipoproteinemia, transmitted as an autosomal dominant trait, there is little, if any, retinal degeneration.(c) Refsum's syndrome is characterized by greatly increased plasma levels of phytanic acid, a 20-carbon branched-chain fatty acid. This substance is not synthesized in the body, but originates from dietary sources only. Patients with Refsum's syndrome lack the enzyme (phytanic acid oxidase) necessary to oxidize this fatty acid, and its accumulation in the tissues has severe consequences. Therapeutic measures, consisting of restriction of dietary phytanic acid, have given encouraging results.(d) Three forms of Batten's disease (neuronal ceroid lipofuscinosis) are now recognized. These are (1) the rapidly progressive (Jansky-Bielschowsky) form, (2) the chronic (Batten-Mayou-Vogt-Spielmeyer-Sjögren) form, which is the most common, and (3) the late onset (Kufs') form. All are associated — to varying degrees — with an accumulation of autofluorescent ceroidlipofuscin pigments. The enzymatic defect is believed to consist of abnormalities in peroxidase enzymes.(e) Other tapetoretinal degenerations thought to be associated with defects in lipid metabolism are discussed briefly. These include Hooffs disease, Cockayne's syndrome, and Alstrom's syndrome.

scholarly journals Anti-Obesity Effects of Collagen Peptide Derived from Skate (Raja kenojei) Skin Through Regulation of Lipid Metabolism

Marine Drugs ◽  
2018 ◽  
Vol 16 (9) ◽  
pp. 306 ◽  
Author(s):  
Minji Woo ◽  
Yeong Song ◽  
Keon-Hee Kang ◽  
Jeong Noh
Keyword(s):  
Body Weight ◽  
Adipose Tissue ◽  
Fatty Acid ◽  
Lipid Metabolism ◽  
Protein Expression ◽  
The Body ◽  
Chow Diet ◽  
Dependent Manner ◽  
Hepatic Lipid ◽  
Collagen Peptide

This study investigated the anti-obesity effects of collagen peptide derived from skate skin on lipid metabolism in high-fat diet (HFD)-fed mice. All C57BL6/J male mice were fed a HFD with 60% kcal fat except for mice in the normal group which were fed a chow diet. The collagen-fed groups received collagen peptide (1050 Da) orally (100, 200, or 300 mg/kg body weight per day) by gavage, whereas the normal and control groups were given water (n = 9 per group). The body weight gain and visceral adipose tissue weight were lower in the collagen-fed groups than in the control group (p < 0.05). Plasma and hepatic lipid levels were significantly reduced by downregulating the hepatic protein expression levels for fatty acid synthesis (sterol regulatory element binding protein-1 (SREBP-1), fatty acid synthase (FAS), and acetyl-CoA carboxylase (ACC)) and cholesterol synthesis (SREBP-2 and 3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR)) and upregulating those for β-oxidation (peroxisome proliferator-activated receptor alpha (PPAR-α) and carnitine palmitoyltransferase 1 (CPT1)) and synthesis of bile acid (cytochrome P450 family 7 subfamily A member 1 (CYP7A1)) (p < 0.05). In the collagen-fed groups, the hepatic protein expression level of phosphorylated 5′ adenosine monophosphate-activated protein kinase (p-AMPK) and plasma adiponectin levels were higher, and the leptin level was lower (p < 0.05). Histological analysis revealed that collagen treatment suppressed hepatic lipid accumulation and reduced the lipid droplet size in the adipose tissue. These effects were increased in a dose-dependent manner. The findings indicated that skate collagen peptide has anti-obesity effects through suppression of fat accumulation and regulation of lipid metabolism.

scholarly journals Perilipin 5 S155 phosphorylation by PKA is required for the control of hepatic lipid metabolism and glycemic control

2020 ◽  
pp. jlr.RA120001126
Author(s):  
Stacey N Keenan ◽  
William DeNardo ◽  
Jieqiong Lou ◽  
Ralf B. Schittenhelm ◽  
Magdalene K. Montgomery ◽  
...  
Keyword(s):  
Fatty Acid ◽  
Lipid Metabolism ◽  
Glycemic Control ◽  
Fatty Acid Oxidation ◽  
Lipid Droplet ◽  
Critical Role ◽  
The Body ◽  
Acid Oxidation ◽  
Hepatic Lipid Metabolism ◽  
Hepatic Lipid

Perilipin (PLIN) 5 is a lipid droplet-associated protein that coordinates intracellular lipolysis in highly oxidative tissues and is thought to regulate lipid metabolism in response to phosphorylation by protein kinase A (PKA). We sought to identify PKA phosphorylation sites in PLIN5 and assess their functional relevance in cultured cells and the livers of mice. We detected phosphorylation on S155, S161 and S163 of recombinant PLIN5 by PKA in vitro and identified S155 as a functionally important site for lipid metabolism. Expression of phosphorylation-defective PLIN5 S155A in Plin5 null cells resulted in decreased rates of lipolysis and triglyceride-derived fatty acid oxidation compared with cells expressing wildtype PLIN5. These differences in lipid metabolism were not associated with differences in the cellular distribution of PLIN5. Rather, FLIM-FRET analysis of protein-protein interactions showed that PLIN5 S155 phosphorylation regulates PLIN5 interaction with adipose triglyceride lipase (ATGL) at the lipid droplet, but not with the co-activator of ATGL, α-β hydrolase domain-containing 5 (ABHD5). Re-expression of PLIN5 S155A in the liver of Plin5 liver-specific null mice reduced lipolysis when compared to mice with wildtype PLIN5 re-expression, but was not associated with other changes in hepatic lipid metabolism, such as fatty acid oxidation, de novo lipogenesis and triglyceride secretion. Furthermore, glycemic control was impaired in mice with expression of PLIN5 S155A compared with mice expressing PLIN5. Together, these studies demonstrate that PLIN5 S155 is required for PKA-mediated lipolysis and builds on the body of evidence demonstrating a critical role for PLIN5 in coordinating lipid and glucose metabolism

Lipids as Triggering Factors in Thrombosis

1976 ◽  
Vol 35 (01) ◽  
pp. 032-048 ◽  
Author(s):  
Arne Nordøy
Keyword(s):  
Lipid Metabolism ◽  
Dietary Habits ◽  
Plasma Lipids ◽  
Experimental Studies ◽  
Plasma Lipid ◽  
High Ratio ◽  
The Body ◽  
Dietary Fats ◽  
Western World ◽  
Platelet Factor

SummaryAn association has been established between acute and more persistent changes in lipid metabolism as reflected in plasma lipids, and platelet lipid metabolism. Platelet function is affected, particularly the activity and availability of platelet factor 3, however, also other changes making the platelets more sensitive to aggregating substances without interfering with the lipid part of platelet factor 3, have been documented. Experimental studies have demonstrated an increased tendency to thrombosis in animals given a diet with a high fat content with a high ratio of saturated to polyunsaturated fatty acids. Studies in man have mainly established a connection between dietary fats, plasma lipid abnormalities and frequency of coronary heart disease and clinical studies more directly relating thrombosis to lipid metabolism is highly warranted. Many open questions remain to be answered. Probably most relevant would be to understand how the antithrombotic mechanisms in the body are affected by changes in lipid metabolism. Even if thrombotic lesions are very common events in the western world our knowledge based on laboratory and experimental studies should indicate a much higher incidence, solely based on interactions between lipids and platelets in subjects exposed to our dietary habits and our way of life.

scholarly journals Effects of Chitosan Oligosaccharide on Plasma and Hepatic Lipid Metabolism and Liver Histomorphology in Normal Sprague-Dawley Rats

Marine Drugs ◽  
2020 ◽  
Vol 18 (8) ◽  
pp. 408 ◽  
Author(s):  
Shing-Hwa Liu ◽  
Rui-Yi Chen ◽  
Meng-Tsan Chiang
Keyword(s):  
Lipid Metabolism ◽  
Normal Control ◽  
Plasma Lipids ◽  
Control Diet ◽  
The Body ◽  
Chitosan Oligosaccharide ◽  
Sprague Dawley ◽  
Hepatic Lipid Metabolism ◽  
Hepatic Lipid ◽  
Sprague Dawley Rats

Chitosan oligosaccharide is known to ameliorate hypercholesterolemia and diabetes. However, some studies found that chitosan oligosaccharide might induce mild to moderate hepatic damage in high-fat (HF) diet-induced obese rats or diabetic rats. Chitosan oligosaccharide can be as a dietary supplement, functional food, or drug. Its possible toxic effects to normal subjects need to be clarified. This study is designed to investigate the effects of chitosan oligosaccharide on plasma and hepatic lipid metabolism and liver histomorphology in normal Sprague-Dawley rats. Diets supplemented with 5% chitosan oligosaccharide have been found to induce liver damage in HF diet-fed rats. We therefore selected 5% chitosan oligosaccharide as an experimental object. Rats were divided into: a normal control diet group and a normal control diet +5% chitosan oligosaccharide group. The experimental period was 12 weeks. The results showed that supplementation of 5% chitosan oligosaccharide did not significantly change the body weight, food intake, liver/adipose tissue weights, plasma lipids, hepatic lipids, plasma levels of AST, ALT, and TNF-α/IL-6, hepatic lipid peroxidation and anti-oxidative enzyme activities, fecal lipids, and liver histomorphology in normal rats. These findings suggest that supplementation of 5% chitosan oligosaccharide for 12 weeks may not induce lipid metabolism disorder and liver toxicity in normal rats.

Alterations in plasma and tissue lipids associated with obesity and metabolic syndrome

2008 ◽  
Vol 114 (3) ◽  
pp. 183-193 ◽  
Author(s):  
Concepción M. Aguilera ◽  
Mercedes Gil-Campos ◽  
Ramón Cañete ◽  
Ángel Gil
Keyword(s):  
Metabolic Syndrome ◽  
Fatty Acid ◽  
Lipid Metabolism ◽  
Fatty Acid Oxidation ◽  
Plasma Lipids ◽  
Density Lipoprotein ◽  
Cellular Fatty Acid ◽  
Acid Oxidation ◽  
Subsequent Increase ◽  
Peripheral Tissues

The MS (metabolic syndrome) is a cluster of clinical and biochemical abnormalities characterized by central obesity, dyslipidaemia [hypertriglyceridaemia and decreased HDL-C (high-density lipoprotein cholesterol)], glucose intolerance and hypertension. Insulin resistance, hyperleptinaemia and low plasma levels of adiponectin are also widely related to features of the MS. This review focuses on lipid metabolism alterations associated with the MS, paying special attention to changes in plasma lipids and cellular fatty acid oxidation. Lipid metabolism alterations in liver and peripheral tissues are addressed, with particular reference to adipose and muscle tissues, and the mechanisms by which some adipokines, namely leptin and adiponectin, mediate the regulation of fatty acid oxidation in those tissues. Activation of the AMPK (AMP-dependent kinase) pathway, together with a subsequent increase in fatty acid oxidation, appear to constitute the main mechanism of action of these hormones in the regulation of lipid metabolism. Decreased activation of AMPK appears to have a role in the development of features of the MS. In addition, alteration of AMPK signalling in the hypothalamus, which may function as a sensor of nutrient availability, integrating multiple nutritional and hormonal signals, may have a key role in the appearance of the MS.

scholarly journals OR-004 Changes in serum indexes of obese adolescents induced by closed weight loss summer camp

2018 ◽  
Vol 1 (2) ◽  
Author(s):  
Chunyan Li ◽  
Caifeng Mao ◽  
Lei Xu ◽  
Feihu Feng
Keyword(s):  
Weight Loss ◽  
Fatty Acid ◽  
Lipid Metabolism ◽  
Oxidative Damage ◽  
Antioxidant Capacity ◽  
Summer Camp ◽  
The Body ◽  
Inflammatory Factors ◽  
Obese Adolescents ◽  
Inflammatory State

Objective A series of experiments were conducted to explore the changes of some serum indexes in obese adolescents induced by closed weight loss summer camp. Methods The 12 to 18 year old obese adolescents (BMI ≥ 28), who volunteered to participate in the Haoqian summer camp, were selected for 4 weeks of closed summer camp. The main activities of the summer camp included compound exercise (aerobic exercise + resistance exercise, 3 times / day, 6 days / week), Dietary intervention, fun activities and health knowledge lectures. In order to explore the changes of serum indexes of obese adolescents, glycolipid metabolism index, fatty acid components, inflammatory factors and oxidative stress markers were analyzed before and after 4 weeks. Results (1) The 4 week weight loss summer camp had no significant effect on blood sugar, but it can obviously reduce the level of serum total cholesterol, triglyceride and low density lipoprotein cholesterol, and significantly improve the abnormal lipid metabolism. (2) The level of serum total saturated fatty acid (P < 0.05), total monounsaturated fatty acid (P < 0.01) and total polyunsaturated fatty acid (P < 0.05) in obese adolescents were decreased significantly in the 4 week weight loss summer camp. (3) The 4 week weight loss summer camp significantly reduced serum inflammatory factors IL-6 and TN F- alpha in obese adolescents, increased the level of adiponectin per body fat mass (P < 0.05), and relieved the inflammatory state of the body. (4) After 4 weeks weight loss summer camp, the serum total antioxidant capacity T-AOC, antioxidant enzyme catalase CAT, superoxide dismutase SOD and glutathione peroxidase GPx activity in obese adolescents were significantly enhanced (P < 0.05); oxidative damage markers 8-iso-PGF2α, 8-OHdG, and MDA levels were not significantly changed (P > 0.05), while protein oxidation product protein carbohydrate PC content decreased significantly (P < 0.05). Conclusions 4 weeks weight loss summer camp can significantly alleviate the body's lipid metabolism abnormalities, change the serum fatty acid components, reduce the body's inflammatory state, enhance the body's antioxidant capacity, and reduce the body's oxidative damage.

Lipid metabolism during lactation: a review of adipose tissue-liver interactions and the development of fatty liver

2005 ◽  
Vol 72 (4) ◽  
pp. 460-469 ◽  
Author(s):  
Richard G Vernon
Keyword(s):  
Type 2 Diabetes ◽  
Fatty Acids ◽  
Adipose Tissue ◽  
Fatty Acid ◽  
Lipid Metabolism ◽  
Negative Energy ◽  
The Body ◽  
Unesterified Fatty Acid ◽  
Adipose Tissue Depots

Fatty acids are the major source of energy for most tissues during periods of negative energy balance; however, fatty acids can, in some circumstances, have pathological effects. Fatty acids are stored as triacylglycerols (TAG), mostly in the various adipose tissue depots of the body. However, if blood unesterified fatty acid (NEFA) levels are elevated for prolonged periods, as may occur during lactation or obesity, TAG can accumulate in other tissues including liver and muscle cells (myocytes), and this can have pathological consequences such as the development of ketosis (Grummer, 1993; Drackley et al. 2001) or type 2 diabetes (Boden & Shulman, 2002; McGarry, 2002).

scholarly journals Evaluation of γ-oryzanol Accumulation and Lipid Metabolism in the Body of Mice Following Long-Term Administration of γ-oryzanol

Nutrients ◽  
2019 ◽  
Vol 11 (1) ◽  
pp. 104 ◽  
Author(s):  
Eri Kobayashi ◽  
Junya Ito ◽  
Naoki Shimizu ◽  
Takumi Kokumai ◽  
Shunji Kato ◽  
...  
Keyword(s):  
Lipid Metabolism ◽  
Plasma Lipids ◽  
Rice Bran Oil ◽  
The Body ◽  
Biologically Active ◽  
Lipid Lowering ◽  
Single Oral Administration ◽  
Term Administration

γ-Oryzanol (OZ), abundant in rice bran oil, has gained attention due to its physiological activities (e.g., lipid-lowering effects). However, the absorption and metabolism of orally ingested OZ have not yet been fully elucidated. In this study, diets containing normal or high contents of OZ were fed to obesity model mice for 8 weeks, and OZ concentrations in plasma and organs were analyzed by HPLC-MS/MS. To evaluate the relationship between OZ accumulation and lipid metabolism in vivo, lipid concentrations in the mice plasma and liver were also measured. As a result, the accumulation of intact OZ in plasma and organs was seen in mice fed diets containing OZ, where mice fed diets containing higher OZ contents demonstrated higher levels of OZ accumulation and lower amounts of plasma lipids. These results, in combination with our additional data from a single oral administration test, suggest the possibility that intact OZ, along with its metabolites (e.g., ferulic acid), is biologically-active.

scholarly journals Association ofn-3 andn-6 long-chain polyunsaturated fatty acids in plasma lipid classes with inflammatory bowel diseases

2007 ◽  
Vol 97 (6) ◽  
pp. 1154-1161 ◽  
Author(s):  
Mária Figler ◽  
Beata Gasztonyi ◽  
Judit Cseh ◽  
Gábor Horváth ◽  
Andrea G. Kisbenedek ◽  
...  
Keyword(s):  
Fatty Acid Composition ◽  
Fatty Acid ◽  
Acid Composition ◽  
Plasma Lipids ◽  
Plasma Lipid ◽  
Lipid Classes ◽  
Biochemical Basis ◽  
Chain Pufa ◽  
Inflammatory Bowel ◽  
Long Chain

In order to establish the biochemical basis for dietary interventions, we investigated the fatty acid composition of plasma lipid classes in patients with inactive inflammatory bowel disease. In this cross-sectional study thirty patients with ulcerative colitis (UC), twenty-one with Crohn disease (CD) and twenty-four controls were investigated (mean age: UC, 40·8 (sd12·1); CD, 37·6 (sd11·0); control, 31·5 (sd8·4) years). Fatty acid composition of plasma lipids was determined by high-resolution capillary GLC. In plasma phospholipids, significantly higher values of eicosapentaenoic (20 : 5n-3), docosapentaenoic (22 : 5n-3) and γ-linolenic (18 : 3n-6) acids were found in control patients and patients with UC as compared to patients with CD [median % (weight by weight), controlv.UCv.CD : 20 : 5n-3, 0·09 (interquartile range (IQR) 0·05)v.0·14 (IQR 0·10)v.0·16 (IQR 0·10),P < 0·05; 22 : 5n-3, 0·14 (IQR 0·10)v.0·27 (IQR 0·16)v.0·31 (IQR 0·10),P < 0·001; 18 : 3n-6, 0·02 (IQR 0·02)v.0·03 (IQR 0·02)v.0·05 (IQR 0·03),P < 0·05]. When compared to the control, values of the principaln-3 andn-6 long-chain PUFA, arachidonic acid (20 : 4n-6) and DHA (22 : 6n-3) were significantly higher in patients with UC but not in patients with CD [median % (w/w), UCv.control: 20 : 4n-6, 8·43 (IQR 3·23)v.6·92 (IQR 2·96),P < 0·05; 22 : 6n-3, 1·22 (IQR 0·56)v.0·73 (IQR 0·39),P < 0·05]. As seen there are considerable differences between the long-chain PUFA status of patients suffering from UC or CD. The data obtained in the present study do not support the concept of eicosapentaenoic acid or DHA deficiency in patients with either UC or CD.

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