Multiple biotin-dependent carboxylase deficiencies associated with defects in T-cell and B-cell immunity. Lancet. 1979; 2 ( 8134):115–8. [ PubMed ]Cowan MJ, Wara DW, Packman S, Yoshino M, Sweetman L & Nyhan W (1979) Multiple biotin-dependent carboxylase deficiencies associated with ...
检测生物素水平的可靠指标是白细胞中的生物素修饰化的甲基巴豆酰辅酶A羧化酶（methylcrotonyl-CoA carboxylase，MCC）和丙酰辅酶A羧化酶（propionyl-CoA carboxylase）。正常成年人的血清生物素浓度应在133-329 pmol/L，尿液中的生物素浓度为18-127 nmol/24小时。确保摄入充足的生物素对大多数人来说并不...
Biotin-Responsive Carboxylase Deficiency Associated with Subnormal Plasma and Urinary Biotin — NEJM 喜欢 0 阅读量: 23 作者:J Thoene,H Baker,M Yoshino,L Sweetman 摘要: The constellation of seizures, lactic acidosis, alopecia, ataxia, and keratoconjunctivitis appears to be pathognomonic for a ...
Metabolic pathway of biotin using different precursors. The precursors synthesized by the BioC/BioH, BioW/BioI and BioZ pathways are indicated with light green, pinkish, and light blue backgrounds.pykA: pyruvate kinase;pykF: pyruvate kinase;pflB: formate C-acetyltransferase;ppc: phosphoenolpyruvate ca...
Biotin-dependent carboxylases function in fatty acid metabolism, gluconeogenesis, and amino acid metabolism. Biotin deficiency is rare in humans; it has been reported only in patients supported by total parenteral nutrition without biotin supplementation, in infants consuming milk with insufficient biotin,...
Biotin-dependent carboxylases play essential roles in fatty acid synthesis, m... J Zempleni,KE Wei,MP Singh,... 被引量: 1发表: 2012年 Biochemistry of biotin Bone metastasis is a deadly complication of cancers arising from many different primary tumor locations. Cross talk between cancer and ...
Prenatal administration of biotin in biotin responsive multiple carboxylase deficiency. Pediatr Res. 1982 Feb; 16 (2):126–129.Roth KS, Yang W, Allan L, Saunders M, Gravel RA, Dakshinamurti K. Prenatal administration of biotin in biotin responsive multiple carboxylase deficiency. Pediatr Res. ...
Pyruvate carboxylase (PC) is a biotin-dependent enzyme that plays a crucial role in gluconeogenesis, lipogenesis, Krebs cycle anaplerosis and amino acid catabolism. Biotin deficiency reduces its mass besides its activity. Enzyme mass is the result of its cellular turnover, i.e., its rates of sy...
Biotin-dependent carboxylases play essential roles in fatty acid synthesis, m... Preedy,R Victor 被引量: 0发表: 2012年 The Chemistry and Biochemistry of Biotin This chapter contains sections titled K Hofmann - John Wiley & Sons, Inc. 被引量: 0发表: 2006年 The structure and function of ...
In mammals, biotin serves as coenzyme for four carboxylases, which play essential roles in the metabolism of glucose, amino acids, and fatty acids. Biotin deficiency causes decreased rates of cell proliferation, impaired immune function, and abnormal fetal development. Evidence is accumulating that b...