Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036474 (scurvy)
 685 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The Osteogenic Disorder Shionogi (ODS) rat, Clea Inc., Tokyo, Japan lacks the ability to synthesize L-ascorbic acid (AA). As with man, monkey and the guinea pig, this rat lacks L-gulonolactone oxidase necessary for the synthesis of AA from glucose. This study shows this animal to be an alternative to the guinea pig in AA studies. The anti-scorbutic potency of Ester C (EC), a calcium ascorbate and calcium threonate mixture, was compared with an AA dose of equal ascorbate activity equivalents (AAE) for anti-scorbutic activity in the ODS rat. The minimal anti-scorbutic dose of EC was determined to be 0.44 mg/kg/day (AAE), while an AA dose of 0.51 mg/kg/day (AAE) was not anti-scorbutic in a 24 day study. At 24 days EC rats gained 125% of initial body weight (BW) and the AA rats only 45% BW. Scorbutic signs at 24 days were scored on a 0 (min) to 3 (max) scale. The EC/AA ratio scores were: hemorrhage 0/1.4, behavior change 0/2.0, piloerection 0/2.2, mobility 0.4/2.2, dysbasia 0.6/2.8 and ataxia 0.4/1.0. Pearson's correlation coefficient for BW versus AAE was r = .34 for the AA group and r = .90 for the EC group. The morbidity index for EC was 0/5 and for the AA group 2/5. The AAE dose of AA which was 16% higher/day than the EC AAE dose was not anti-scorbutic, while the EC dose was anti-scorbutic. EC rats had 3.5X greater weight gain, a sensitive indicator of scurvy, than the AA rats. EC rats had 3-4 times less, if any, scorbutic signs than AA rats. The results clearly show that, based on ascorbate activity equivalents, EC has more available ascorbate activity/potency than AA. The mechanism of this increased potency is believed to be due to the facilitated transport of AAE into the cell by the threonate (a normal in vivo metabolite of AA) present in the EC product. In addition, previous studies have shown EC (AAE) to be higher in plasma and excreted less rapidly than the AAE derived from AA administered orally.
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PMID:Comparison of the anti-scorbutic activity of L-ascorbic acid and Ester C in the non-ascorbate synthesizing Osteogenic Disorder Shionogi (ODS) rat. 190 22

1. The effect of ascorbic acid deficiency on glycosaminoglycans of granulation tissue and cartilage of guinea pigs was investigated by determination of the changes in the glucosamine and galactosamine contents 12 days after tendonectomy. 2. In normal granulation tissue, the glucosamine and galactosamine contents rose to a peak at 5 and 10 days respectively, whereas the hydroxyproline and proline contents continued to rise throughout the 20 days after tendonectomy. 3. The galactosamine in scorbutic granulation tissue, but not in that of pair-fed controls, decreased significantly in absolute amount and relatively to glucosamine, which remained practically unchanged; the cartilage galactosamine did not decrease during the 22 days of deficiency owing to the presence of excess of preformed galactosaminoglycans, which masked the small amount of newly formed glycosaminoglycans. 4. The chemical results were confirmed by radioactivity studies in vivo of incorporation of [U-(14)C]glucose into galactosamine and glucosamine of scorbutic granulation tissue and cartilage. The incorporation of (14)C into galactosamine decreased significantly in scurvy in both tissues. 5. The results indicated in both tissues a decreased formation of galactosamine during scurvy, although an increased degradation of polymerized glycosaminoglycans could not be entirely ruled out. It is concluded that, if lack of ascorbic acid causes an impaired galactosamine formation, the most likely position for the block may be in the UDP-N-acetylglucosamine 4-epimerase reaction.
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PMID:The effect of scurvy on glycosaminoglycans of granulation tissue and costal cartilage. 423 17

Latent scurvy is characterized by a reversible atherosclerosis that closely resembles the clinical form of this disease. Acute scurvy is characterized by microvascular complications such as widespread capillary hemorrhaging. Vitamin C (ascorbate) is required for the synthesis of collagen, the protein most critical in the maintenance of vascular integrity. We suggest that in latent scurvy, large blood vessels use modified LDL--in particular lipoprotein(a)--in addition to collagen to maintain macrovascular integrity. By this mechanism, collagen is spared for the maintenance of capillaries, the sites of gas and nutrient exchange. The foam-cell phenotype of atherosclerosis is identified as a mesenchymal genetic program, regulated by the availability of ascorbate. When vitamin C is limited, foam cells develop and induce oxidative modification of LDL, thereby stabilizing large blood vessels via the deposition of LDL. The structural similarity between vitamin C and glucose suggests that hyperglycemia will inhibit cellular uptake of ascorbate, inducing local vitamin C deficiency.
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PMID:Hyperglycemia-induced latent scurvy and atherosclerosis: the scorbutic-metaplasia hypothesis. 869 35

The pattern of fruit juice consumption has changed over time. Fifty years ago, orange juice was the major juice produced and it was consumed primarily to prevent scurvy. Now, apple juice is the juice of choice for the under 5 age group. While fruit juice is a healthy, low-fat, nutritious beverage, there have been some health concerns regarding juice consumption. Nursing bottle caries have long been recognized as a consequence of feeding juice in bottles, using the bottle as a pacifier, and prolonged bottle feeding. Non-specific chronic diarrhea or "toddler's" diarrhea has been associated with juice consumption, especially juices high in sorbitol and those with a high fructose to glucose ratio. This relates to carbohydrate malabsorption, which varies by the type, concentration, and mixture of sugars present in different fruit juices. Fruit juice consumption by preschoolers has recently increased from 3.2 to about 5.5 fl oz/day. Consumption of fruit juice helps fulfill the recommendation to eat more fruits and vegetables, with fruit juice accounting for 50% of all fruit servings consumed by children, aged 2 through 18 years, and 1/3 of all fruits and vegetables consumed by preschoolers. Concomitant with the increase in fruit juice consumption has been a decline in milk intake. This is concerning as milk is the major source of calcium in the diet, and at present, only 50% of children, aged 1 through 5 years, meet the RDA for calcium. Studies of newborn infants and preschool-aged children have demonstrated a preference for sweet-tasting foods and beverages. Thus, it is not surprising that some children, if given the opportunity, might consume more fruit juice than is considered optimal. Eleven percent of healthy preschoolers consumed > or = 12 fl oz/day of fruit juice, which is considered excessive. Excess fruit juice consumption has been reported as a contributing factor in some children with nonorganic failure to thrive and in some children with decreased stature. In other children, excessive fruit juice consumption has been associated with an increased caloric intake and obesity. This paper reviews the role of fruit juice in the diets of infants and children and outlines areas for future research. Recommendations regarding fruit juice consumption based on current data are also given.
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PMID:Fruit juice consumption by infants and children: a review. 889 77

The reviewed studies addressed the possibility of using gene transfer for correction of L-ascorbic acid biosynthesis and carbohydrate utilization in rainbow trout. Analyses of enzymatic activities in the L-AAB pathway indicated that reasons for the lack of L-AA production can be common in fish and scurvy-prone animals. Rat gulonolactone oxidase cDNA was transferred into trout. Regardless of the fact that rGLO transcription occurred in embryos, neither GLO protein, nor enzyme activity were detected. There was no production of L-AA in transgenic fish raised on vitamin C-free diets or injected with L-gulonolactone. These results indicated that the conditions required for translation or stability of rGLO were not present in trout tissues. To augment carbohydrates utilization, human glucose transporter 1 and rat hexokinase II cDNAs were tested. In the transfected embryos. HK activity, rates of hexose uptake and glucose oxidation were increased. The effect of hGLUT1 on glucose metabolism was greater than that of rHKII. Trout carrying hGLUT1 and rHKII with viral or piscine promoters were created. Though interpretation of the metabolic effects of the transgenes was complicated with mosaicism, a tendency to improved carbohydrate utilization was revealed in some of the transgenic individuals.
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PMID:Gene transfer for targeted modification of salmonid fish metabolism. 1059 49

Vitamin C is an essential dietary nutrient required as a co-factor for many enzymes, and humans are among the few animals that lack the ability to synthesize the compound from glucose. The reduced form of the vitamin, ascorbic acid, is an especially effective antioxidant owing to its high electron-donating power and ready conversion back to the active reduced form. Concentrations of the vitamin in body tissues and fluids are regulated through interactions of intestinal absorption, cellular transport, and excretion. The amount of vitamin C needed to prevent scurvy is very small and easily obtained in nearly all Western diets. There is great interest in the clinical roles of vitamin C because of evidence that oxidative damage is a root cause of, or at least associated with, many diseases. Population studies show that individuals with high intakes of vitamin C have lower risk of a number of chronic diseases, including heart disease, cancer, eye diseases, and neurodegenerative conditions. However, these results may simply reflect a more healthful diet or lifestyle for individuals with a high vitamin C intake. At present, data from controlled clinical trials have not established that higher intakes of vitamin C alone will help prevent chronic degenerative diseases. However, the evidence that ascorbic acid acts as an important antioxidant in many body tissues is convincing. The new higher Recommended Dietary Allowance (RDA) for vitamin C of 75 mg for women and 90 mg for men is, for the first time, based on the vitamin's role as an antioxidant as well as protection from deficiency. In healthy people, amounts greater than the RDA do not appear to be helpful. Vitamin C nutriture may be more important for people with certain diseases or conditions. High intakes of the vitamin are generally well tolerated; a Tolerable Upper Level was recently set at 2 g based on gastrointestinal upset that sometimes accompanies excessive intakes.
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PMID:Vitamin C function and status in chronic disease. 1213 9

Vitamin C or ascorbic acid is a hydrosoluble vitamin derived from glucose metabolism. It acts as a reductor agent required for synthesis of collagen fibers through hydroxylation of proline and lysine. It also protects the body against damage caused by the free radicals. Humans cannot synthesize ascorbic acid as they lack an enzyme called gulonolactone oxidase. Concentrations in plasma and leukocytes reflect the levels of the diet and body deposits respectively of this vitamin. Among foods with high vitamin C levels are tomatoes, potatoes, and citrus fruits such as limes, oranges and lemons. The current recommendation of daily intake of vitamin C is 90 mg/d for men and 75 mg/d for women. Patients with chronic diseases such as cancer or diabetes or those who smoke need higher doses in their usual diet. Ascorbic acid deficiency gives rise to the appearance of scurvy. This disease is rarely seen in developed countries. The symptoms develop with plasma levels below 0.15 mg/dL. Scurvy is characterized by the presence of weakness, joint pain or skin lesions in form of petechias, gum bleeding, ease of developing bruises or delay in wound healing. The most characteristic skin manifestations are purpuric perifollicular hyperkeratotic papules and the presence of kinky hair.
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PMID:[Vitamin C]. 1717 58

Ascorbate (vitamin C) deficiency leads to low immunity, scurvy, and other human diseases and is therefore a global health problem. Given that plants are major ascorbate sources for humans, biofortification of this vitamin in our foodstuffs is of considerable importance. Ascorbate is synthetized by a number of alternative pathways: (i) from the glycolytic intermediates D-glucose-6P (the key intermediates are GDP-D-mannose and L-galactose), (ii) from the breakdown of the cell wall polymer pectin which uses the methyl ester of D-galacturonic acid as precursor, and (iii) from myo-inositol as precursor via myo-inositol oxygenase. We report here the engineering of fruit-specific overexpression of a bacterial pyrophosphatase, which hydrolyzes the inorganic pyrophosphate (PPi) to orthophosphate (Pi). This strategy resulted in increased vitamin C levels up to 2.5-fold in ripe fruit as well as increasing in the major sugars, sucrose, and glucose, yet decreasing the level of starch. When considered together, these finding indicate an intimate linkage between ascorbate and sugar biosynthesis in plants. Moreover, the combined data reveal the importance of PPi metabolism in tomato fruit metabolism and development.
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PMID:Pyrophosphate levels strongly influence ascorbate and starch content in tomato fruit. 2395 Jul 59