Biotin deficiency effect

The physiologic sequelae of biotin deficiency are almost unexplored. Severe skin lesions, especially seborrheic dermatitis and Leiner s disease (Erythroderma desquamativum or exfoliative dermatitis), were increased in young infants bom of mothers on a restricted diet low in eggs, livers, and other biotin-rich foods. After biotin administration the lesions healed. There are claims that excess biotin produces a fatty liver characterized by heightened cholesterol content. Choline has no effect in the prevention of biotin-fatty livers (G2, M2). In mice with transplanted tumors, both the tumors and the blood levels of biotin are below normal (R8). More recent studies established a protection with avidin, the biotin-binding fraction of egg white, against tumor formation (K4). More data along these lines are still needed for confirmation. 

Patients with complete biotinidase deficiency usually present between 3 and 6 months of age, but severe illness has already been noted at the 2nd and 3rd week of life [9]. On the contrary, patients defined as having profound biotinidase deficiency, who have levels of residual activity as low as 1-3%, may never develop obvious clinical symptoms [17, 33], but may suffer from moderate biotin deficiency when carefully evaluated [25]. Biotin deficiency can be effectively avoided by oral... 

Figure 6. Effect of sucrose on abnormal glutamate accumulation observed in biotin-deficient cells of L. arabinosus...

Bannister DW (1976b) Hepatic gluconeogenesis in chicks effect of biotin on gluconeogenesis in biotin-deficiency and fatty liver and kidney syndrome. Comparative Biochemistry and Physiology B 53, 575-9. 

Mock DM, Johnson SB, and ffolman RT (f988a) Effects of biotin deficiency on serum fatty acid composition evidence for abnormalities in humans. Journal of Nutrition 118,342-8. 

Biotin Deficiency. Relative to many of the vitamins, it is easy to induce a biotin deficiency by feeding volunteers raw egg white. Avidin, a basic protein found in egg white, forms salt linkages with acidic biotin and prevent its transport across the intestinal barrier. Cooked egg white is not a problem. Because biotin is found in the yolk, eating whole raw egg will not induce a deficiency. Deficiencies also were caused in patients on total parenteral nutrition (TPN) because biotin was not included in the early formulations. Symptoms include dermatitis, loss of hair color, and central neurological effects. 

Polymorphic plates from water, mp 238 (some dec). [a]Jf — 39.5 (c = 1.01 in 0.1 IV NaOH). fneffective in curing the biotin deficiency syndrome when administered to rats at a level 100 times the effective dose of biotin. 

Amino acid deaminases, IX, 36-38 biotin and, IX, 38-44 Amino acid deficiency, effect on gonads, X, 96-98 Amino acid oxidase (s), action of, IX, 36 biotin and, IX, 54 effect on biotin, X, 337 flavoprotein nature of, IX, 36 in tissues, steroid hormones and, X, 367 D-Amino acid oxidase, effect of adrenalectomy on, X, 320 of steroid hormones on, X, 319, 320, 352-358... 

Terroine (1954) has examined the effect of ascorbic acid on biotin deficiency in rats. She used lower levels than in her later experiments on thiamine and riboflavin deficiencies. She found that 0.1-0.5% ascorbic acid only slightly improved growth and appetite but considerably reduced the incidence of signs of biotin deficiency. Thus, in 92 days, all the deficient animals had developed alopecia, but only 30% of those receiving ascorbic acid. Similarly, 90% of the deficient group had spectacle eyes, but none... 

All aspects of the biochemical role of biotin have not yet been clarified. The vitamin has been implicated in the metabolism of carbohydrates, lipids, proteins, and nucleic acids. Available evidence indicates that biotin acts as a CO2 carrier in a number of carboxyla-tion and decarboxylation reactions connected with carbohydrate and fatty acid metabolism. A number of experimental procedures are used to establish the participation of biotin in a given biochemical reaction (1) the study of enzyme activity in biotin-deficient animals (2) the effect of avidin administered in vivo or added to the incubation mixture on the activity of the enzyme under study and (3) purification of the enzyme and demonstration of the existence of enzyme-bound biotin. Studies of this kind have established that biotin is required for the carboxylases of jS-methyl-crotonyl CoA, acetyl-CoA, propionyl CoA, and oxaloacetic transcarboxylase. Only some of the results are presented here [74-76]. 

A few years later egg-white injury in chicks was shown to be associated with a deficiency of biotin in the tissues, despite its abundance in the diet [145]. In the same year, avidin was isolated from egg-white and its ability to inactivate biotin in vitro was demonstrated [146]. Gyorgy and Rose [235] fed rats with avidin but only found very small amounts of biotin in the faeces until the faeces were steamed. Biotin was then released from the avidin-biotin complex. As the result of further experiments, it was concluded that the fundamental cause of egg-white injury is the unavailability of biotin due to its fixation to avidin, so that biotin is not absorbed from the intestinal tract and is excreted in the faeces [237]. A similar conclusion was reached by Sullivan and Nicholls [603] who showed that when egg-white is cooked, avidin is denatured and rendered incapable of binding biotin. Egg-white injury has been produced experimentally in man and can be cured by the administration of biotin [607]. In a recent study, Peters [497] reported that raw egg-white has a direct toxic effect which is not associated with its action in causing biotin deficiency. It would appear, therefore, that further studies on egg-white injury must be more closely associated with a critical analysis of the different components of egg-white. 

J.M.Peters, A Separation of the Direct Toxic Effects of Dietary Raw Egg White Powder from Its Action in Producing Biotin Deficiency, Br. J. Nutr. 21, 801-809... 

Suchy, S.F., Rizzo, W.B., and Wolf, B., 1986. Effect of biotin deficiency and supplementation on lipid metabohsm in rats saturated fatty acids. The American Journal of Clinical Nutrition. 44 475-480. 

Biotin is synthesized by many microorganisms and plants and is an essential nutrient for higher organisms. The recognition of biotin-responsive multiple carboxylase deficiency syndrome and its treatment with biotin has been a major contribution to the understanding of neonatal disease states (5,6). The effects of experimental biotin deficiency in a variety of species are quite devastating. 

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