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4. Acetyl CoA carboxylase in cultured fibroblasts: differential biotin dependence in the two types of biotin-responsive multiple carboxylase deficiency. Packman S; Caswell N; Gonzalez-Rios MC; Kadlecek T; Cann H; Rassin D; McKay C Am J Hum Genet; 1984 Jan; 36(1):80-92. PubMed ID: 6141728 [TBL] [Abstract][Full Text] [Related]
5. Heterogeneity of holocarboxylase synthetase in patients with biotin-responsive multiple carboxylase deficiency. Burri BJ; Sweetman L; Nyhan WL Am J Hum Genet; 1985 Mar; 37(2):326-37. PubMed ID: 3920902 [TBL] [Abstract][Full Text] [Related]
6. Biotin holocarboxylase synthetase: purification from rat liver cytosol and some properties. Xia WL; Zhang J; Ahmad F Biochem Mol Biol Int; 1994 Sep; 34(2):225-32. PubMed ID: 7849632 [TBL] [Abstract][Full Text] [Related]
7. Lactic acidosis in biotin-responsive multiple carboxylase deficiency caused by holocarboxylase synthetase deficiency of early and late onset. Sherwood WG; Saunders M; Robinson BH; Brewster T; Gravel RA J Pediatr; 1982 Oct; 101(4):546-50. PubMed ID: 6811711 [TBL] [Abstract][Full Text] [Related]
8. Biotin-responsive multiple carboxylase deficiency in an 8-year-old boy with normal serum biotinidase and fibroblast holocarboxylase-synthetase activities. Holme E; Jacobson CE; Kristiansson B J Inherit Metab Dis; 1988; 11(3):270-6. PubMed ID: 3148068 [TBL] [Abstract][Full Text] [Related]
9. Deficiency of biotinyl-AMP synthetase activity in fibroblasts of patients with holocarboxylase synthetase deficiency. Morita J; Thuy LP; Sweetman L Mol Genet Metab; 1998 Aug; 64(4):250-5. PubMed ID: 9758715 [TBL] [Abstract][Full Text] [Related]
10. Evidence for multiple forms of biotin holocarboxylase synthetase in pea (Pisum sativum) and in Arabidopsis thaliana: subcellular fractionation studies and isolation of a cDNA clone. Tissot G; Douce R; Alban C Biochem J; 1997 Apr; 323 ( Pt 1)(Pt 1):179-88. PubMed ID: 9173880 [TBL] [Abstract][Full Text] [Related]
11. Clustering of mutations in the biotin-binding region of holocarboxylase synthetase in biotin-responsive multiple carboxylase deficiency. Dupuis L; Leon-Del-Rio A; Leclerc D; Campeau E; Sweetman L; Saudubray JM; Herman G; Gibson KM; Gravel RA Hum Mol Genet; 1996 Jul; 5(7):1011-6. PubMed ID: 8817339 [TBL] [Abstract][Full Text] [Related]
12. Protein biotinylation in higher plants: characterization of biotin holocarboxylase synthetase activity from pea (Pisum sativum) leaves. Tissot G; Job D; Douce R; Alban C Biochem J; 1996 Mar; 314 ( Pt 2)(Pt 2):391-5. PubMed ID: 8670045 [TBL] [Abstract][Full Text] [Related]
14. Five patients with a biotin-responsive defect in holocarboxylase formation: evaluation of responsiveness to biotin therapy in vivo and comparative biochemical studies in vitro. Suormala T; Fowler B; Duran M; Burtscher A; Fuchshuber A; Tratzmüller R; Lenze MJ; Raab K; Baur B; Wick H; Baumgartner R Pediatr Res; 1997 May; 41(5):666-73. PubMed ID: 9128289 [TBL] [Abstract][Full Text] [Related]
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17. Management of a patient with holocarboxylase synthetase deficiency. Van Hove JL; Josefsberg S; Freehauf C; Thomas JA; Thuy le P; Barshop BA; Woontner M; Mock DM; Chiang PW; Spector E; Meneses-Morales I; Cervantes-Roldán R; León-Del-Río A Mol Genet Metab; 2008 Dec; 95(4):201-5. PubMed ID: 18974016 [TBL] [Abstract][Full Text] [Related]
18. Holocarboxylase Synthetase: A Moonlighting Transcriptional Coregulator of Gene Expression and a Cytosolic Regulator of Biotin Utilization. León-Del-Río A; Valadez-Graham V; Gravel RA Annu Rev Nutr; 2017 Aug; 37():207-223. PubMed ID: 28564555 [TBL] [Abstract][Full Text] [Related]
19. Enzymatic diagnosis of holocarboxylase synthetase deficiency using apo-carboxyl carrier protein as a substrate. Suzuki Y; Aoki Y; Sakamoto O; Li X; Miyabayashi S; Kazuta Y; Kondo H; Narisawa K Clin Chim Acta; 1996 Jul; 251(1):41-52. PubMed ID: 8814349 [TBL] [Abstract][Full Text] [Related]
20. Biotin regulates the genetic expression of holocarboxylase synthetase and mitochondrial carboxylases in rats. Rodríguez-Meléndez R; Cano S; Méndez ST; Velázquez A J Nutr; 2001 Jul; 131(7):1909-13. PubMed ID: 11435506 [TBL] [Abstract][Full Text] [Related] [Next] [New Search]