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

Riboflavin-ultraviolet A (UVA) collagen cross-linking has not only achieved good clinical efficacy in the treatment of corneal diseases such as dilatation keratopathy, bullae keratopathy, infectious keratopathy, and in the combined treatment of corneal refractive surgeries, but also its efficacy and safety in scleral collagen cross-linking have been initially confirmed. To better promote the application of cross-linking in the clinical treatment of corneal and scleral diseases, exploring controllability and predictability of the surgical efficacy are both important for evaluating the surgical efficacy and personalized precision treatment. In this paper, the progress on the cross-linking depth of riboflavin-UVA collagen cross-linking, and its relationship with the cross-linking effect will be reviewed. It will provide the reference for further application of this procedure in ophthalmology clinics.
Riboflavin/pharmacology* ; Humans ; Collagen/radiation effects* ; Ultraviolet Rays ; Cross-Linking Reagents ; Corneal Diseases/drug therapy* ; Photosensitizing Agents/therapeutic use*

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

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

Cornea ; Epithelium, Corneal ; Iontophoresis ; Riboflavin

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

BACKGROUND: The correlations between the amount of daily fiber intake and bone mineral densities (BMDs) in Korean adult population were investigated in our study. METHODS: Utilizing the Korean National Health and Nutrition Examination Survey in 2011, multivariable linear regression was performed to explore the association between fiber consumption and BMD of lumbar vertebrae 1 to 4 (L1–4 total), L1, L2, L3, and L4 vertebrae, femur neck, femur total, and trochanter. All models were adjusted for age, body mass index, vitamin D level, smoking, physical activity, alcohol use, contraceptive use, hormonal replacement therapy, consumption of carbohydrate, protein, fat, calcium, phosphate, iron, thiamine, riboflavin, niacin, and vitamin C. RESULTS: In males aged between 18 and 45, fiber intake significantly increased BMDs of L1 (coefficient β=0.004, P=0.040) and L2 (β=0.004, P=0.038) while daily protein consumption significantly lowered BMDs of femur neck (β=−0.001, P=0.009), femur total (β=−0.001, P=0.008), and trochanter (β=−0.001, P=0.008). In males aged 65 and older, nutrient intake shows no significant correlations with BMDs except fat consumption was inversely associated with BMD of trochanter (β=−0.001, P=0.017). In females aged between 18 and 45, fiber intake shows no significant relationship with BMDs while daily fat consumption significantly increased BMDs of L1 (β=0.001, P=0.028), L2 (β=0.001, P=0.024), L3 (β=0.001, P=0.033), and L1–4 total (β=0.001, P=0.017). CONCLUSIONS: Fiber intake was a protective factor of lumbar spine (L1 and L2) BMD in male aged between 18 and 45 but not in female participants of any age groups.
Adult ; Ascorbic Acid ; Body Mass Index ; Bone Density ; Calcium ; Dietary Fiber ; Female ; Femur ; Femur Neck ; Humans ; Iron ; Linear Models ; Lumbar Vertebrae ; Male ; Motor Activity ; Niacin ; Nutrition Surveys ; Osteoporosis ; Protective Factors ; Riboflavin ; Smoke ; Smoking ; Spine ; Thiamine ; Vitamin D

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