Lipid storage myopathy （LSM） is a lipid metabolic disorder characterized by excessive lipid droplet accumulation in muscle fibers. Classic multiple Acyl-CoA dehydrogenase deficiency （MADD）， also known as glutaric aciduria type Ⅱ， is a disease with various clinical manifestations caused by mutations in electron transfer flavoprotein （ETF） and ETF-ubiquinone oxidoreductase. In recent years， a large amount of evidence has shown that classic late-onset MADD caused by mutations in the electron transfer flavoprotein dehydrogenase gene is the main cause of LSM. Besides classic MADD， many other diseases with similar changes in blood acylcarnitines and urinary organic acids can also cause LSM， and such diseases are call MADD-like disorders or MADD spectrum. This article reviews the clinical， pathological， biochemical， and molecular features of LSM with various etiologies and the latest advances in treatment， with a focus on the latest findings associated with MADD spectrum.
Riboflavin

This study aims to investigate the mechanism of Linderae Radix water extract(LRWE) in the prevention and treatment of diarrhea-predominant irritable bowel syndrome(IBS-D) based on serum metabolomics. Eighteen 2-week-old male SD rats were randomized into control, IBS-D model, and LRWE groups. The rats in other groups except the control group received gavage of senna concentrate combined with restraint stress for the modeling of IBS-D. The rats in the LRWE group were administrated with LRWE(5.4 g·kg~(-1)) by gavage, and those in the control and IBS-D model groups with an equal volume of distilled water for a total of 14 days. The visceral sensitivity was evaluated by the abdominal withdrawal reflex(AWR) score, and the degree of diarrhea was assessed by the fecal water content(FWC). The morphological changes of the colon and the morphology and number of goblet cells were observed by hematoxylin-eosin(HE) and periodic acid-schiff(PAS) staining, respectively. Ultra-high performance liquid chromatography-tandem mass spectrometry(UPLC-MS/MS) was used for the screening of the potential biomarkers in the rat serum and their related metabolic pathways. The results showed that LRWE reduced the AWR score, decreased FWC, and alleviated visceral sensitivity and diarrhea symptoms in IBS-D rats. HE and PAS staining showed that LRWE mitigated low-grade intestinal inflammation and increased the number of mature secretory goblet cells in the colonic epithelium of IBS-D rats. A total of 25 potential biomarkers of LRWE in treating IBS-D were screened out in this study, which were mainly involved in riboflavin, tryptophan, glycine, serine and threonine metabolism, glyoxylate and dicarboxylate metabolism, and cysteine and methionine metabolism. The regulatory effects were the most significant on the riboflavin and tryptophan metabolism pathways. LRWE may alleviate the visceral hypersensitivity by promoting energy metabolism and amino acid metabolism, enhancing intestinal barrier function, and improving intestinal immune function in IBS-D rats.
Rats ; Male ; Animals ; Irritable Bowel Syndrome/metabolism* ; Water ; Chromatography, Liquid ; Tryptophan ; Rats, Sprague-Dawley ; Tandem Mass Spectrometry ; Diarrhea/drug therapy* ; Biomarkers ; Riboflavin

Cornea ; Epithelium, Corneal ; Iontophoresis ; Riboflavin

Flavoproteins are proteins that contain a nucleic acid derivative of riboflavin: flavin adenine dinucleotide (FAD) or flavin mononucleotide (FMN). Flavoproteins are involved in a wide array of biological processes, such as photosynthesis, DNA repair and natural product biosynthesis. It should be noted that 5%-10% of flavoproteins have a covalently linked flavin prosthetic group. Such covalent linkages benefit the holoenzyme in several ways including improving the stability and catalytic potency. During the past decade, significant progress has been made in covalent flavoproteins, especially with respect to enzyme-dependent biogenesis and discovery of novel linkage types. The present review gives a condensed overview of investigations published from March 2009 to December 2021, with emphasis on the discovery, biogenesis and their catalytic role in natural product biosynthesis.
Flavoproteins/metabolism* ; Flavin-Adenine Dinucleotide/metabolism* ; Flavin Mononucleotide/metabolism* ; Riboflavin ; Biological Products

The effect of parasitic ions on the results of ultraviolet A (UVA) cross-linking in iontophoresis was still not clear. In this work, the porcine sclera was cross-linked by riboflavin lactate Ringer's solution (group A) and riboflavin normal saline (group B)
Animals ; Collagen ; Cross-Linking Reagents ; Ions ; Iontophoresis ; Permeability ; Photosensitizing Agents/pharmacology* ; Riboflavin ; Sclera ; Swine ; Ultraviolet Rays

China is the country with high incidence of high myopia in the world. High myopia can cause severe vision impairment. So far, there is no effective treatment for high myopia in clinic. Scleral collagen cross-linking surgery has been proven to be effective in preventing animal eye axial elongation
Animals ; Collagen ; Cross-Linking Reagents ; Finite Element Analysis ; Photosensitizing Agents ; Riboflavin ; Sclera

riboflavin, niacin, vitamin B₁₂, pantothenic acid, sodium, animal iron, zinc, selenium, and cholesterol (negative trends), and carbohydrate rate, fiber, water-soluble fiber, vitamin K, vitamin C, and plant calcium (positive trends). Multivariate analysis revealed that animal fat, animal iron, and niacin (negative trends) and animal protein, fiber, and vitamin C (positive trends) were statistically significant. Pantothenic acid and sodium showed non-significant negative trends (P < 0.1), and vitamin B₁₂ showed a non-significant positive trend.CONCLUSION: This study provided a nutritional basis and extended the utility of ACFS, which is a bridgehead for future cancer-preventive clinical trials using ACFS.]]>
Animals ; Ascorbic Acid ; Asian Continental Ancestry Group ; Calcium ; Cholesterol ; Diet ; Humans ; Iron ; Multivariate Analysis ; Niacin ; Pantothenic Acid ; Plants ; Riboflavin ; Selenium ; Sodium ; Vegetarians ; Vitamin D ; Vitamin K ; Vitamins ; Zinc

Ophiopogon japonicus is one of the commonly used medicines,and it has gradually become a therapeutic food for people's daily health care. O. japonicus in Sichuan province is the famous Dao-di herbs in Sichuan province,and is mainly produced in Santai county,Sichuan province. With the unique geographical advantage,Santai county plans to declare the new food raw materials of O. japonicus based on the geographical indication products( Fu Cheng O. japonicus),so it is necessary to analyze and evaluate the nutritional components of O. japonicus in Santai county. The experimental results showed that the content of the nutrients was characterized by low fat,high polysaccharide,high potassium and high vitamin B2,which can be developed as new food raw materials.
China ; Drugs, Chinese Herbal ; Nutrients ; analysis ; Nutritive Value ; Ophiopogon ; chemistry ; Plant Roots ; chemistry ; Polysaccharides ; analysis ; Potassium ; analysis ; Riboflavin ; analysis

BACKGROUND AND OBJECTIVES: This study aimed to analyze risk factors for positional vertigo (PV) and the influence thereof on daily life and subjective quality-of-life (QoL). SUBJECT ANDMETHODS: A cross-sectional study was conducted using data of the 2010 Korea National Health and Nutrition Examination Survey. The study population consisted of 1,274 individuals aged >40 years for whom complete dizziness-related data were available. Blood and urine tests were performed, and nutritional intake, QoL, and subjective health status were measured using a questionnaire. The associations between PV and blood/urine test data and nutritional intake were evaluated via multiple logistic regression analysis. RESULTS: A history of PV within the previous year was reported by 98 individuals (7.7%). Advanced age and female gender were both significantly associated with such a history. Serum hemoglobin, creatinine, and triglyceride levels correlated significantly with a history of PV. Carotene, vitamin A, and vitamin B2 intakes were significantly lower in individuals with PV. Multivariate analysis revealed that only age was significantly associated with a history of PV (p=0.003). Although subjective health status score was not significantly lower in subjects with PV, subjective impairments in mobility, self-care, the performance of usual activities, and anxiety/depression were more prominent in individuals with PV. A fall history and limitations of activity were also significantly higher in individuals with PV (p < 0.001 and p=0.003, respectively). CONCLUSIONS: Age was a risk factor for PV, which affected most QoL parameters, fall frequency, and the performance of normal activities.
Carotenoids ; Creatinine ; Cross-Sectional Studies ; Diagnostic Self Evaluation ; Dizziness ; Female ; Humans ; Korea ; Logistic Models ; Multivariate Analysis ; Nutrition Surveys ; Riboflavin ; Risk Factors ; Self Care ; Triglycerides ; Vertigo ; Vitamin A

BACKGROUND: The health condition of old age is affected by various factors such as economic level, disease condition, and nutrition. With the aging population in Korea, the ratio of single-person households increased rapidly. Research on the health status and nutrition of the elderly in the single-person household is very insufficient. In this study, we compared the health and nutritional status of the elderly by the household type. METHODS: Data from the 2013 to 2014 Korea National Health and Nutrition Examination Survey were used. A total of 2,730 patients were classified into 2 groups (single-person, with family), and general, chronic disease, health behavior, nutrient intake, and food insecurity status were compared by the statistical analysis. RESULTS: Single-person households had a low economic and educational level and a higher percentage of women. In addition, obesity, hypertension, dyslipidemia, stroke, myocardial infarction disease rate was significantly higher. Sing-person households answered that their subjective health status was bad, and their quality of life was low. As a result of analysis of the quality of the diet in the single-person, the intake of protein, calcium, iron, vitamin B2, niacin, and vitamin C was significantly lower. In particular, the intake of calcium was the most insufficient. Food insecurity has also been observed, including the inability to consume diverse and sufficient foods due to economic difficulties. CONCLUSIONS: More attention should be paid to the health of single-person households in elderly population and various policies should be prepared.
Aged ; Aging ; Ascorbic Acid ; Calcium ; Chronic Disease ; Diagnostic Self Evaluation ; Diet ; Dyslipidemias ; Family Characteristics ; Female ; Food Supply ; Health Behavior ; Humans ; Hypertension ; Iron ; Korea ; Myocardial Infarction ; Niacin ; Nutrition Surveys ; Nutritional Status ; Obesity ; Quality of Life ; Riboflavin ; Single Person ; Stroke