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Animal : An International Journal of... Jan 2021Alternatives to riboflavin (vitamin B) production by recombinant microorganisms are needed in organic poultry production, but are cost-intensive, so that a...
Alternatives to riboflavin (vitamin B) production by recombinant microorganisms are needed in organic poultry production, but are cost-intensive, so that a demand-oriented riboflavin supply is necessary. Details on the riboflavin requirements of organic poultry are not available. A feed material with high native riboflavin content from fermentation of the filamentous fungus Ashbya gossypii was studied. Two runs with 800 Ranger Gold™ broilers each (40 pens with 20 animals) were conducted. The fattening period was divided into starter (S), grower (G) and finisher (F) stage. In the first run, a basal diet without riboflavin supplementation (NATIVE; 3.27, 3.50 and 3.16 mg riboflavin/kg DM in S, G and F) was compared to diets with supplementation at low (LOW; 5.30, 4.85 and 5.19 mg/kg in S, G and F), medium (MEDIUM; 7.56, 6.88 and 7.56 mg/kg in S, G and F) and high (HIGH; 10.38, 9.14 and 9.93 mg/kg in S, G and F) dosage. In the second run, different combinations of low and medium riboflavin supplementation were used in S, G and F diets: S-LOW (4.50 mg riboflavin/kg DM), G-MEDIUM (6.66 mg/kg), F-MEDIUM (5.71 mg/kg) (Treatment A), S-LOW (4.50 mg riboflavin/kg DM); G-LOW (4.92 mg/kg), F-LOW (4.01 mg/kg) (Treatment B); S-MEDIUM (6.37 mg/kg), G-MEDIUM (7.37 mg/kg), F-MEDIUM (5.07 mg/kg) (Treatment C); S-MEDIUM (6.37 mg/kg), G-LOW (5.28 mg/kg), F-LOW (4.22 mg/kg) (Treatment D). Body weight, feed and water consumption were recorded weekly, health and welfare indicators were scored bi-weekly. Slaughter traits were assessed for five males and females per pen. In the first run, NATIVE animals showed symptoms of riboflavin deficiency and lower live weights in the second week of age. Riboflavin contents of this group were increased to avoid further deficiency and recovery was observed. Feed conversion was better in HIGH (2.07) compared with NATIVE and LOW (2.11). At slaughter, treatments differed neither for foot pad dermatitis nor plumage cleanliness. In the second run, daily weight gains did not differ between treatments in any of the weeks. Feed conversion ranged between 1.99 and 2.04. Riboflavin deficiency was not observed in the second run, while treatment D showed superior economic efficiency. In conclusion, native contents of feed components (3.27 mg/kg DM) were not sufficient to meet the riboflavin demand and a total content of 4.50 mg/kg DM was identified as safe lower threshold. The levels rather according to commercial recommendations were not additionally beneficial to performance and health.
Topics: Animal Feed; Animal Nutritional Physiological Phenomena; Animals; Chickens; Diet; Eremothecium; Female; Fermentation; Male; Riboflavin
PubMed: 33516011
DOI: 10.1016/j.animal.2020.100003 -
Advances in Nutrition (Bethesda, Md.) May 2012Infants should be exclusively breastfed for the first 6 mo of life. However, maternal deficiency of some micronutrients, conveniently classified as Group I... (Review)
Review
Infants should be exclusively breastfed for the first 6 mo of life. However, maternal deficiency of some micronutrients, conveniently classified as Group I micronutrients during lactation, can result in low concentrations in breast milk and subsequent infant deficiency preventable by improving maternal status. This article uses thiamin, riboflavin, vitamin B-6, vitamin B-12, and choline as examples and reviews the evidence for risk of inadequate intakes by infants in the first 6 mo of life. Folate, a Group II micronutrient, is included for comparison. Information is presented on forms and concentrations in human milk, analytical methods, the basis of current recommended intakes for infants and lactating women, and effects of maternal supplementation. From reports of maternal and/or infant deficiency, concentrations in milk were noted as well as any consequences for infant function. These milk values were used to estimate the percent of recommended daily intake that infants fed by a deficient mother could obtain from her milk. Estimates were 60% for thiamin, 53% for riboflavin, 80% for vitamin B-6, 16% for vitamin B-12, and 56% for choline. Lack of data limits the accuracy and generalizability of these conclusions, but the overall picture that emerges is consistent across nutrients and points to an urgent need to improve the information available on breast milk quality.
Topics: Breast Feeding; Choline; Dietary Supplements; Female; Folic Acid; Humans; Infant; Lactation; Maternal Nutritional Physiological Phenomena; Milk, Human; Nutritional Status; Riboflavin; Vitamin B 12; Vitamin B 6; Vitamin B Complex; Vitamin B Deficiency
PubMed: 22585913
DOI: 10.3945/an.111.001172 -
Orphanet Journal of Rare Diseases Oct 2012The Brown-Vialetto-Van Laere syndrome is a rare neurological disorder which may present at all ages with sensorineural deafness, bulbar palsy and respiratory compromise.... (Review)
Review
The Brown-Vialetto-Van Laere syndrome is a rare neurological disorder which may present at all ages with sensorineural deafness, bulbar palsy and respiratory compromise. Fazio-Londe syndrome is considered to be the same disease entity. Recently it was demonstrated that in some patients the disease is caused by mutations in the SLC52A3 gene which encodes the intestinal (hRFT2) riboflavin transporter. In these patients riboflavin deficiency is the cause of the BVVL/FL syndrome and supplementation of riboflavin proved a life saving treatment. Mutations in the SLC52A2 gene and the SLC52A1 (GPR172B) gene, coding for human riboflavin transporters hRFT3 and hRFT1 have been associated with the BVVL syndrome as well. We performed a review of the literature, with emphasis on the natural history and the effects of treatment in these patients. A total of 35 publications were traced reporting on the clinical presentation of 74 patients who presented before age 18. The most prevalent symptoms were bulbar palsy, hearing loss, facial weakness and respiratory compromise. Death was reported in 28 of the 61 untreated patients, with a very low survival in patients presenting before age 4. All 13 patients who were treated with riboflavin survived, with a strong clinical improvement after days to months of treatment in eight patients. Three patients demonstrated a stable clinical course and treatment was stopped early in two patients. Abnormalities in plasma flavin levels and/or plasma acylcarnitine profiles were observed in some but not in all patients, and also patients with normal plasma flavin levels and acylcarnitine profiles demonstrated a striking clinical improvement on riboflavin supplementation. It is now clear that proper diagnosis requires mutation analysis of all three transporter genes and treatment should be started immediately without first awaiting results of molecular analysis. Clinical improvement may be rapid or gradual over a period of more than 12 months.
Topics: Bulbar Palsy, Progressive; Female; Hearing Loss, Sensorineural; Humans; Male; Riboflavin
PubMed: 23107375
DOI: 10.1186/1750-1172-7-83 -
JIMD Reports Jul 2022In this report, we describe the case of an 11-year-old boy, who came to our attention for myalgia and muscle weakness, associated with inappetence and vomiting....
In this report, we describe the case of an 11-year-old boy, who came to our attention for myalgia and muscle weakness, associated with inappetence and vomiting. Hypertransaminasemia was also noted, with ultrasound evidence of hepatomegaly. Biochemical investigations revealed acylcarnitine and organic acid profiles resembling those seen in MADD, that is, multiple acyl-CoA dehydrogenase deficiencies (OMIM #231680) a rare inherited disorder of fatty acids, amino acids, and choline metabolism. The patient carried a single pathogenetic variant in the gene (c.524G>A, p.Arg175His) and no pathogenetic variant in the riboflavin (Rf) homeostasis related genes (, , , , ). Instead, compound heterozygosity was found in the gene (c.512C>G, p.Ser171Cys; c.822C>A, p.Asn274Lys), coding for isobutyryl-CoA dehydrogenase (IBD), whose pathogenic variants are associated to IBD deficiency (OMIM #611283), a rare autosomal recessive disorder of valine catabolism. The c.822C>A was never previously described in a patient. Subsequent further analyses of Rf homeostasis showed reduced levels of flavins in plasma and altered FAD-dependent enzymatic activities in erythrocytes, as well as a significant reduction in the level of the plasma membrane Rf transporter 2 in erythrocytes. The observed Rf/flavin scarcity in this patient, possibly associated with a decreased ETF:QO efficiency might be responsible for the observed MADD-like phenotype. The patient's clinical picture improved after supplementation of Rf, l-carnitine, Coenzyme Q10, and also 3OH-butyrate. This report demonstrates that, even in the absence of genetic defects in genes involved in Rf homeostasis, further targeted molecular analysis may reveal secondary and possibly treatable biochemical alterations in this pattern.
PubMed: 35822092
DOI: 10.1002/jmd2.12292 -
Nutrients Dec 2014A recent Korea National Health and Nutrition Survey indicated inadequate riboflavin intake in Koreans, but there is limited research regarding riboflavin status in South...
A recent Korea National Health and Nutrition Survey indicated inadequate riboflavin intake in Koreans, but there is limited research regarding riboflavin status in South Korea. The purpose of this study was to determine riboflavin intake and status of Korean adults. Three consecutive 24-h food recalls were collected from 412 (145 men and 267 women) healthy adults, aged 20-64 years, living in South Korea and urine samples were collected from 149 subjects of all subjects. The dietary and total (dietary plus supplemental) riboflavin intake was 1.33 ± 0.34 and 2.87 ± 6.29 mg/day, respectively. Approximately 28% of the subjects consumed total riboflavin less than the Estimated Average Requirement. Urinary riboflavin excretion was 205.1 ± 190.1 μg/g creatinine. Total riboflavin intake was significantly positively correlated to the urinary riboflavin excretion. (r = 0.17171, p = 0.0363). About 11% of the Korean adults had urinary riboflavin <27 μg/g creatinine indicating a riboflavin deficiency and 21% had low status of riboflavin (27 μg/g creatinine ≤ urinary riboflavin < 80 μg/g creatinine). Thus, one-third of Korean adults in this study had inadequate riboflavin status. In some adults in Korea, consumption of riboflavin-rich food sources should be encouraged.
Topics: Adult; Creatinine; Dietary Supplements; Feeding Behavior; Female; Humans; Male; Mental Recall; Middle Aged; Nutrition Surveys; Nutritional Requirements; Republic of Korea; Riboflavin; Riboflavin Deficiency; Young Adult
PubMed: 25558909
DOI: 10.3390/nu7010253 -
Journal of the Formosan Medical... Apr 2020Atrophic glossitis (AG) is characterized by the partial or complete absence of filiform papillae on the dorsal surface of the tongue. AG may reflect the significant... (Review)
Review
Atrophic glossitis (AG) is characterized by the partial or complete absence of filiform papillae on the dorsal surface of the tongue. AG may reflect the significant deficiencies of some major nutrients including riboflavin, niacin, pyridoxine, vitamin B12, folic acid, iron, zinc, and vitamin E. Moreover, protein-calorie malnutrition, candidiasis, Helicobacter pylori colonization, xerostomia, and diabetes mellitus are also the etiologies of AG. Our previous study found the serum gastric parietal cell antibody (GPCA), thyroglobulin antibody (TGA), and thyroid microsomal antibody (TMA) positivities in 26.7%, 28.4%, and 29.8% of 1064 AG patients, respectively. We also found anemia, serum iron, vitamin B12, and folic acid deficiencies, and hyperhomocysteinemia in 19.0%, 16.9%, 5.3%, 2.3%, and 11.9% of 1064 AG patients, respectively. Moreover, GPCA-positive AG patients tended to have relatively higher frequencies of hemoglobin, iron, and vitamin B12 deficiencies and hyperhomocysteinemia than GPCA-negative AG patients. Supplementations with vitamin BC capsules plus corresponding deficient hematinics for those AG patients with hematinic deficiencies can achieve complete remission of oral symptoms and AG in some AG patients. Therefore, it is very important to examine the complete blood count, serum hematinic, homocysteine, and autoantibody levels in AG patients before we start to offer treatments for AG patients.
Topics: Anemia; Atrophy; Autoantibodies; Erythrocyte Indices; Folic Acid; Folic Acid Deficiency; Glossitis; Hemoglobins; Humans; Hyperhomocysteinemia; Iron; Parietal Cells, Gastric; Vitamin B 12; Vitamin B 12 Deficiency
PubMed: 31076315
DOI: 10.1016/j.jfma.2019.04.015 -
Orphanet Journal of Rare Diseases Jan 2024Multiple acyl-CoA dehydrogenase deficiency (MADD) is an autosomal recessive disorder resulting from pathogenic variants in three distinct genes, with most of the...
BACKGROUND
Multiple acyl-CoA dehydrogenase deficiency (MADD) is an autosomal recessive disorder resulting from pathogenic variants in three distinct genes, with most of the variants occurring in the electron transfer flavoprotein-ubiquinone oxidoreductase gene (ETFDH). Recent evidence of potential founder variants for MADD in the South African (SA) population, initiated this extensive investigation. As part of the International Centre for Genomic Medicine in Neuromuscular Diseases study, we recruited a cohort of patients diagnosed with MADD from academic medical centres across SA over a three-year period. The aim was to extensively profile the clinical, biochemical, and genomic characteristics of MADD in this understudied population.
METHODS
Clinical evaluations and whole exome sequencing were conducted on each patient. Metabolic profiling was performed before and after treatment, where possible. The recessive inheritance and phase of the variants were established via segregation analyses using Sanger sequencing. Lastly, the haplotype and allele frequencies were determined for the two main variants in the four largest SA populations.
RESULTS
Twelve unrelated families (ten of White SA and two of mixed ethnicity) with clinically heterogeneous presentations in 14 affected individuals were observed, and five pathogenic ETFDH variants were identified. Based on disease severity and treatment response, three distinct groups emerged. The most severe and fatal presentations were associated with the homozygous c.[1067G > A];c.[1067G > A] and compound heterozygous c.[976G > C];c.[1067G > A] genotypes, causing MADD types I and I/II, respectively. These, along with three less severe compound heterozygous genotypes (c.[1067G > A];c.[1448C > T], c.[740G > T];c.[1448C > T], and c.[287dupA*];c.[1448C > T]), resulting in MADD types II/III, presented before the age of five years, depending on the time and maintenance of intervention. By contrast, the homozygous c.[1448C > T];c.[1448C > T] genotype, which causes MADD type III, presented later in life. Except for the type I, I/II and II cases, urinary metabolic markers for MADD improved/normalised following treatment with riboflavin and L-carnitine. Furthermore, genetic analyses of the most frequent variants (c.[1067G > A] and c.[1448C > T]) revealed a shared haplotype in the region of ETFDH, with SA population-specific allele frequencies of < 0.00067-0.00084%.
CONCLUSIONS
This study reveals the first extensive genotype-phenotype profile of a MADD patient cohort from the diverse and understudied SA population. The pathogenic variants and associated variable phenotypes were characterised, which will enable early screening, genetic counselling, and patient-specific treatment of MADD in this population.
Topics: Humans; Child, Preschool; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; Mutation; South Africa; Genotype; Riboflavin; Guanine Nucleotide Exchange Factors; Death Domain Receptor Signaling Adaptor Proteins
PubMed: 38221620
DOI: 10.1186/s13023-023-03014-8 -
Journal of the Academy of Nutrition and... May 2020Riboflavin, vitamin B-6, and vitamin B-12 are key players in one-carbon metabolism as enzymatic cofactors, and deficiency of these nutrients may influence reproductive...
BACKGROUND
Riboflavin, vitamin B-6, and vitamin B-12 are key players in one-carbon metabolism as enzymatic cofactors, and deficiency of these nutrients may influence reproductive outcomes possibly through affecting reproductive hormones.
OBJECTIVE
The goal was to investigate associations between dietary intakes of riboflavin, vitamin B-6, and vitamin B-12, and menstrual function among premenopausal women.
DESIGN
This was a secondary analysis of a prospective cohort study conducted at the University at Buffalo during 2005 to 2007.
PARTICIPANTS/SETTING
Participants were 259 healthy, regularly menstruating women (aged 18 to 44 years) with self-reported menstrual cycles between 21 and 35 days, who were not trying to conceive, and who had not used hormonal contraception during the past 3 months.
MAIN OUTCOME MEASURES
Intakes of B vitamins were assessed via 24-hour dietary recalls four times per menstrual cycle for two cycles. Serum reproductive hormones and plasma homocysteine were measured eight and three times, respectively, per cycle for two cycles. Anovulatory cycles were determined by progesterone concentrations ≤5 ng/mL (15.9 nmol/L) and no observed serum luteinizing hormone peak during the mid or late luteal phase visit.
STATISTICAL ANALYSIS
Weighted linear mixed regressions were used to evaluate associations between cycle-averaged B vitamin intakes and hormones and homocysteine, and generalized linear regressions for associations with anovulation. Models were adjusted for age, race, body mass index, physical activity, alternate Mediterranean diet score, intakes of total energy, protein, fiber, and folate, and percentage of energy intake from fat.
RESULTS
Higher intakes of riboflavin (per 0.1 mg increase in intake) were inversely correlated with estradiol (-0.87%, 95% CI -1.67 to -0.06) and homocysteine levels (-0.61%, 95% CI -1.10 to -0.12). Higher vitamin B-6 intakes were suggestive of higher follicle-stimulating hormone, although the results were not statistically significant (0.63% difference, 95% CI -0.03 to 1.29, per 0.1 mg increase in intake; P=0.06). Small increases in testosterone and decreases in homocysteine were found with vitamin B-12 intake. No associations were observed between intake of B vitamins and a risk of sporadic anovulation.
CONCLUSIONS
Higher intakes of riboflavin were associated with a small decrease in serum estradiol among healthy, regularly menstruating women. Higher intakes of riboflavin and vitamin B-12 were associated with lower plasma homocysteine concentrations. Overall, riboflavin, vitamin B-6, and vitamin B-12 that are one-carbon nutrients do not appear to influence the ovarian cycle among premenopausal women.
Topics: Adolescent; Adult; Anovulation; Diet Surveys; Eating; Estradiol; Female; Follicle Stimulating Hormone; Healthy Volunteers; Humans; Luteinizing Hormone; Menstrual Cycle; Premenopause; Progesterone; Prospective Studies; Riboflavin; Testosterone; Vitamin B 12; Vitamin B 6; Young Adult
PubMed: 31879178
DOI: 10.1016/j.jand.2019.10.013 -
AoB PLANTS Jun 2021Phosphorus (P) is an essential element for the growth and development of plants. Soybean () is an important food crop that is grown worldwide. Soybean yield is...
Phosphorus (P) is an essential element for the growth and development of plants. Soybean () is an important food crop that is grown worldwide. Soybean yield is significantly affected by P deficiency in the soil. To investigate the molecular factors that determine the response and tolerance at low-P in soybean, we conducted a comparative proteomics study of a genotype with low-P tolerance (Liaodou 13, L13) and a genotype with low-P sensitivity (Tiefeng 3, T3) in a paper culture experiment with three P treatments, i.e. P-free (0 mmol·L), low-P (0.05 mmol·L) and normal-P (0.5 mmol·L). A total of 4126 proteins were identified in roots of the two genotypes. Increased numbers of differentially expressed proteins (DEPs) were obtained from low-P to P-free conditions compared to the normal-P treatment. All DEPs obtained in L13 (660) were upregulated in response to P deficiency, while most DEPs detected in T3 (133) were downregulated under P deficiency. Important metabolic pathways such as oxidative phosphorylation, glutathione metabolism and carbon metabolism were suppressed in T3, which could have affected the survival of the plants in P-limited soil. In contrast, L13 increased the metabolic activity in the 2-oxocarboxylic acid metabolism, carbon metabolism, glycolysis, biosynthesis of amino acids, pentose phosphatase, oxidative phosphorylation, other types of -glycan biosynthesis and riboflavin metabolic pathways in order to maintain normal plant growth under P deficiency. Three key proteins I1KW20 (prohibitins), I1K3U8 (alpha-amylase inhibitors) and C6SZ93 (alpha-amylase inhibitors) were suggested as potential biomarkers for screening soybean genotypes with low-P tolerance. Overall, this study provides new insights into the response and tolerance to P deficiency in soybean.
PubMed: 34150189
DOI: 10.1093/aobpla/plab019 -
Cleveland Clinic Journal of Medicine Oct 2016Certain vitamin and mineral deficiencies may be recognized by their cutaneous signs. This case-based article reviews deficiencies of zinc and vitamins A, B, B, B, and C,... (Review)
Review
Certain vitamin and mineral deficiencies may be recognized by their cutaneous signs. This case-based article reviews deficiencies of zinc and vitamins A, B, B, B, and C, discussing their consequences and skin findings.
Topics: Adolescent; Aged; Ascorbic Acid Deficiency; Female; Humans; Middle Aged; Niacin; Riboflavin Deficiency; Skin Diseases; Vitamin A Deficiency; Vitamin B 6 Deficiency; Zinc
PubMed: 27726828
DOI: 10.3949/ccjm.83a.15061