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Annual Review of Nutrition Aug 2022The consumption of fructose as sugar and high-fructose corn syrup has markedly increased during the past several decades. This trend coincides with the exponential rise... (Review)
Review
The consumption of fructose as sugar and high-fructose corn syrup has markedly increased during the past several decades. This trend coincides with the exponential rise of metabolic diseases, including obesity, nonalcoholic fatty liver disease, cardiovascular disease, and diabetes. While the biochemical pathways of fructose metabolism were elucidated in the early 1990s, organismal-level fructose metabolism and its whole-body pathophysiological impacts have been only recently investigated. In this review, we discuss the history of fructose consumption, biochemical and molecular pathways involved in fructose metabolism in different organs and gut microbiota, the role of fructose in the pathogenesis of metabolic diseases, and the remaining questions to treat such diseases.
Topics: Diet; Fructose; High Fructose Corn Syrup; Humans; Liver; Metabolic Diseases; Non-alcoholic Fatty Liver Disease
PubMed: 35995049
DOI: 10.1146/annurev-nutr-062220-025831 -
Roczniki Panstwowego Zakladu Higieny 2015Sweeteners, both natural and artificial, play an important role in a human diet as well as are of great importance to the food industry and dieticians. Many people... (Review)
Review
Sweeteners, both natural and artificial, play an important role in a human diet as well as are of great importance to the food industry and dieticians. Many people associate sweet taste with sucrose, which is commonly known as table sugar. However, there are many sweet substances that food manufacturers add to food products because none of them is ideal for all applications. Besides sucrose there are also other sugars such as glucose and fructose that originate both from natural sources such as fruits and honey or from added sugars. Among sweeteners there are also compounds which have a sweet taste and contain no calories or those which sweetness is so intense so can be used at very low concentrations, thus, their impact on the total caloric value of the product is negligible. They can be classified due to their origin (natural or synthetic agents), the technological function (sweeteners and fillers), texture (powders and syrups), and nutritional value (caloric and non-caloric). Natural sweetening substances include carbohydrates, sugar alcohols, thaumatin and stevia. Besides providing well tasting foods, they might have an impact on products' texture, color, preservation and caloric value. Sugar alcohols, which belong to carbohydrates, are both natural sugar substitutes and food additives. They are becoming more and more popular among consumers mainly due to their lower caloric values and glycemic indexes as well as anticariogenic effects. Sugar alcohols are often combined with other sweeteners to enhance food products' sweetness. Stevia, which is 200 times sweeter than sucrose, is a non caloric substance whereas thaumatin, a sweet protein, provides 4 kcal/g but characterizes with sweetness about 2000 times higher than sucrose (on a weight basis).
Topics: Diet; Dietary Carbohydrates; Dietary Sucrose; Food Preferences; Health Promotion; Humans; Nutritive Value; Sugar Alcohols; Sweetening Agents
PubMed: 26400114
DOI: No ID Found -
The Application of Clinical Genetics 2017Maple syrup urine disease (MSUD) is an inborn error of metabolism caused by defects in the branched-chain α-ketoacid dehydrogenase complex, which results in elevations... (Review)
Review
Maple syrup urine disease (MSUD) is an inborn error of metabolism caused by defects in the branched-chain α-ketoacid dehydrogenase complex, which results in elevations of the branched-chain amino acids (BCAAs) in plasma, α-ketoacids in urine, and production of the pathognomonic disease marker, alloisoleucine. The disorder varies in severity and the clinical spectrum is quite broad with five recognized clinical variants that have no known association with genotype. The classic presentation occurs in the neonatal period with developmental delay, failure to thrive, feeding difficulties, and maple syrup odor in the cerumen and urine, and can lead to irreversible neurological complications, including stereotypical movements, metabolic decompensation, and death if left untreated. Treatment consists of dietary restriction of BCAAs and close metabolic monitoring. Clinical outcomes are generally good in patients where treatment is initiated early. Newborn screening for MSUD is now commonplace in the United States and is included on the Recommended Uniform Screening Panel (RUSP). We review this disorder including its presentation, screening and clinical diagnosis, treatment, and other relevant aspects pertaining to the care of patients.
PubMed: 28919799
DOI: 10.2147/TACG.S125962 -
Journal of Pediatric Gastroenterology... Dec 2017The consumption of sugars, particularly sugar-sweetened beverages (SSBs; beverages or drinks that contain added caloric sweeteners (ie, sucrose, high-fructose corn... (Review)
Review
The consumption of sugars, particularly sugar-sweetened beverages (SSBs; beverages or drinks that contain added caloric sweeteners (ie, sucrose, high-fructose corn syrup, fruit juice concentrates), in European children and adolescents exceeds current recommendations. This is of concern because there is no nutritional requirement for free sugars, and infants have an innate preference for sweet taste, which may be modified and reinforced by pre- and postnatal exposures. Sugar-containing beverages/free sugars increase the risk for overweight/obesity and dental caries, can result in poor nutrient supply and reduced dietary diversity, and may be associated with increased risk of type 2 diabetes mellitus, cardiovascular risk, and other health effects. The term "free sugars," includes all monosaccharides/disaccharides added to foods/beverages by the manufacturer/cook/consumer, plus sugars naturally present in honey/syrups/unsweetened fruit juices and fruit juice concentrates. Sugar naturally present in intact fruits and lactose in amounts naturally present in human milk or infant formula, cow/goat milk, and unsweetened milk products is not free sugar. Intake of free sugars should be reduced and minimised with a desirable goal of <5% energy intake in children and adolescents aged ≥2 to 18 years. Intake should probably be even lower in infants and toddlers <2 years. Healthy approaches to beverage and dietary consumption should be established in infancy, with the aim of preventing negative health effects in later childhood and adulthood. Sugar should preferably be consumed as part of a main meal and in a natural form as human milk, milk, unsweetened dairy products, and fresh fruits, rather than as SSBs, fruit juices, smoothies, and/or sweetened milk products. Free sugars in liquid form should be replaced by water or unsweetened milk drinks. National Authorities should adopt policies aimed at reducing the intake of free sugars in infants, children and adolescents. This may include education, improved labelling, restriction of advertising, introducing standards for kindergarten and school meals, and fiscal measures, depending on local circumstances.
Topics: Adolescent; Beverages; Cardiovascular Diseases; Child; Child, Preschool; Consensus; Dental Caries; Diabetes Mellitus, Type 2; Dietary Sucrose; Energy Intake; Europe; Food; Food Labeling; Humans; Infant; Pediatric Obesity; Recommended Dietary Allowances; Societies, Medical
PubMed: 28922262
DOI: 10.1097/MPG.0000000000001733 -
Cell Metabolism Dec 2021Glucose and fructose are closely related simple sugars, but fructose has been associated more closely with metabolic disease. Until the 1960s, the major dietary source... (Review)
Review
Glucose and fructose are closely related simple sugars, but fructose has been associated more closely with metabolic disease. Until the 1960s, the major dietary source of fructose was fruit, but subsequently, high-fructose corn syrup (HFCS) became a dominant component of the Western diet. The exponential increase in HFCS consumption correlates with the increased incidence of obesity and type 2 diabetes mellitus, but the mechanistic link between these metabolic diseases and fructose remains tenuous. Although dietary fructose was thought to be metabolized exclusively in the liver, evidence has emerged that it is also metabolized in the small intestine and leads to intestinal epithelial barrier deterioration. Along with the clinical manifestations of hereditary fructose intolerance, these findings suggest that, along with the direct effect of fructose on liver metabolism, the gut-liver axis plays a key role in fructose metabolism and pathology. Here, we summarize recent studies on fructose biology and pathology and discuss new opportunities for prevention and treatment of diseases associated with high-fructose consumption.
Topics: Diabetes Mellitus, Type 2; Fructose; Glucose; High Fructose Corn Syrup; Humans; Liver
PubMed: 34619076
DOI: 10.1016/j.cmet.2021.09.004 -
Molecular Genetics and Metabolism Mar 2020Over the past three decades, we studied 184 individuals with 174 different molecular variants of branched-chain α-ketoacid dehydrogenase activity, and here delineate...
Over the past three decades, we studied 184 individuals with 174 different molecular variants of branched-chain α-ketoacid dehydrogenase activity, and here delineate essential clinical and biochemical aspects of the maple syrup urine disease (MSUD) phenotype. We collected data about treatment, survival, hospitalization, metabolic control, and liver transplantation from patients with classic (i.e., severe; n = 176), intermediate (n = 6) and intermittent (n = 2) forms of MSUD. A total of 13,589 amino acid profiles were used to analyze leucine tolerance, amino acid homeostasis, estimated cerebral amino acid uptake, quantitative responses to anabolic therapy, and metabolic control after liver transplantation. Standard instruments were used to measure neuropsychiatric outcomes. Despite advances in clinical care, classic MSUD remains a morbid and potentially fatal disorder. Stringent dietary therapy maintains metabolic variables within acceptable limits but is challenging to implement, fails to restore appropriate concentration relationships among circulating amino acids, and does not fully prevent cognitive and psychiatric disabilities. Liver transplantation eliminates the need for a prescription diet and safeguards patients from life-threatening metabolic crises, but is associated with predictable morbidities and does not reverse pre-existing neurological sequelae. There is a critical unmet need for safe and effective disease-modifying therapies for MSUD which can be implemented early in life. The biochemistry and physiology of MSUD and its response to liver transplantation afford key insights into the design of new therapies based on gene replacement or editing.
Topics: 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide); Adolescent; Adult; Amino Acids, Branched-Chain; Biomarkers; Child; Child, Preschool; Cognitive Dysfunction; Cohort Studies; Diet; Female; Homozygote; Humans; Infant; Leucine; Liver Transplantation; Male; Maple Syrup Urine Disease; Mental Disorders; Middle Aged; Phenotype
PubMed: 31980395
DOI: 10.1016/j.ymgme.2020.01.006 -
Digestive Diseases and Sciences May 2016Nonalcoholic fatty liver disease (NAFLD) is a liver manifestation of metabolic syndrome. Overconsumption of high-fat diet (HFD) and increased intake of sugar-sweetened... (Review)
Review
Nonalcoholic fatty liver disease (NAFLD) is a liver manifestation of metabolic syndrome. Overconsumption of high-fat diet (HFD) and increased intake of sugar-sweetened beverages are major risk factors for development of NAFLD. Today the most commonly consumed sugar is high fructose corn syrup. Hepatic lipids may be derived from dietary intake, esterification of plasma free fatty acids (FFA) or hepatic de novo lipogenesis (DNL). A central abnormality in NAFLD is enhanced DNL. Hepatic DNL is increased in individuals with NAFLD, while the contribution of dietary fat and plasma FFA to hepatic lipids is not significantly altered. The importance of DNL in NAFLD is further established in mouse studies with knockout of genes involved in this process. Dietary fructose increases levels of enzymes involved in DNL even more strongly than HFD. Several properties of fructose metabolism make it particularly lipogenic. Fructose is absorbed via portal vein and delivered to the liver in much higher concentrations as compared to other tissues. Fructose increases protein levels of all DNL enzymes during its conversion into triglycerides. Additionally, fructose supports lipogenesis in the setting of insulin resistance as fructose does not require insulin for its metabolism, and it directly stimulates SREBP1c, a major transcriptional regulator of DNL. Fructose also leads to ATP depletion and suppression of mitochondrial fatty acid oxidation, resulting in increased production of reactive oxygen species. Furthermore, fructose promotes ER stress and uric acid formation, additional insulin independent pathways leading to DNL. In summary, fructose metabolism supports DNL more strongly than HFD and hepatic DNL is a central abnormality in NAFLD. Disrupting fructose metabolism in the liver may provide a new therapeutic option for the treatment of NAFLD.
Topics: Diet; Fructose; High Fructose Corn Syrup; Humans; Lipogenesis; Liver; Non-alcoholic Fatty Liver Disease
PubMed: 26856717
DOI: 10.1007/s10620-016-4054-0 -
Molecular Genetics and Metabolism Jul 2014In an effort to increase harmonization of care and enable outcome studies, the Genetic Metabolic Dietitians International (GMDI) and the Southeast Regional Newborn... (Meta-Analysis)
Meta-Analysis
In an effort to increase harmonization of care and enable outcome studies, the Genetic Metabolic Dietitians International (GMDI) and the Southeast Regional Newborn Screening and Genetics Collaborative (SERC) are partnering to develop nutrition management guidelines for inherited metabolic disorders (IMD) using a model combining both evidence- and consensus-based methodology. The first guideline to be completed is for maple syrup urine disease (MSUD). This report describes the methodology used in its development: formulation of five research questions; review, critical appraisal and abstraction of peer-reviewed studies and unpublished practice literature; and expert input through Delphi surveys and a nominal group process. This report includes the summary statements for each research question and the nutrition management recommendations they generated. Each recommendation is followed by a standardized rating based on the strength of the evidence and consensus used. The application of technology to build the infrastructure for this project allowed transparency during development of this guideline and will be a foundation for future guidelines. Online open access of the full, published guideline allows utilization by health care providers, researchers, and collaborators who advise, advocate and care for individuals with MSUD and their families. There will be future updates as warranted by developments in research and clinical practice.
Topics: Age Factors; Diet; Dietary Supplements; Disease Management; Evidence-Based Medicine; Female; Health Care Surveys; Humans; Liver Transplantation; Male; Maple Syrup Urine Disease; Practice Guidelines as Topic; Pregnancy
PubMed: 24881969
DOI: 10.1016/j.ymgme.2014.05.006 -
International Journal of Environmental... Jun 2022Agave syrup (AS), a food product made from agave plant sap, is a vegan sweetener that has become popular for replacing conventional sweeteners such as sucrose. As the... (Review)
Review
Agave syrup (AS), a food product made from agave plant sap, is a vegan sweetener that has become popular for replacing conventional sweeteners such as sucrose. As the demand for naturally derived sweeteners has grown in the last decade, this review paper addresses and discusses, in detail, the most relevant aspects of the chemical AS analysis, applications in the food industry, sustainability issues, safety and quality control and, finally, nutritional profile and health impacts. According to our main research outcome, we can assume that the mid-infrared-principal components analysis, high-performance anion exchange chromatography equipped with a pulsed amperometric detector, and thin-layer chromatography can be used to identify and distinguish syrups from natural sources. The main agave-derived products are juice, leaves, bagasse, and fiber. In sustainability terms, it can be stated that certified organic and free trade agave products are the most sustainable options available on the market because they guarantee products being created without pesticides and according to specific labor standards. The Mexican government and AS producers have also established Mexican guidelines which prohibit using any ingredient, sugar or food additive that derives from sources, apart from agave plants, to produce any commercial AS. Due to its nutritional value, AS is a good source of minerals, vitamins and polyphenols compared to other traditional sweeteners. However, further research into the effects of AS on human metabolism is necessary to back its health claims as a natural sugar substitute.
Topics: Agave; Carbohydrates; Food Additives; Food Industry; Humans; Sweetening Agents
PubMed: 35742286
DOI: 10.3390/ijerph19127022