Biotin

⇄

holocarboxylase synthetase ; Homo sapiens







78 Article(s)
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Title
Pub. Year
#Total Relationships
51 14613969 Reduced histone biotinylation in multiple carboxylase deficiency patients: a nuclear role for holocarboxylase synthetase. 2004 Jan 1 3
52 15456772 Paradoxical regulation of biotin utilization in brain and liver and implications for inherited multiple carboxylase deficiency. 2004 Dec 10 3
53 12855220 Partial response to biotin therapy in a patient with holocarboxylase synthetase deficiency: clinical, biochemical, and molecular genetic aspects. 2003 Jul 1
54 11959985 Holocarboxylase synthetase is an obligate participant in biotin-mediated regulation of its own expression and of biotin-dependent carboxylases mRNA levels in human cells. 2002 Apr 16 10
55 12097659 Biotin uptake into human peripheral blood mononuclear cells increases early in the cell cycle, increasing carboxylase activities. 2002 Jul 1
56 12124727 Clinical findings and biochemical and molecular analysis of four patients with holocarboxylase synthetase deficiency. 2002 Jul 22 2
57 11735028 Structure of human holocarboxylase synthetase gene and mutation spectrum of holocarboxylase synthetase deficiency. 2001 Nov 4
58 10653324 Diagnosis and molecular analysis of an atypical case of holocarboxylase synthetase deficiency. 2000 Jan-Feb 4
59 10064313 Molecular biology of biotin attachment to proteins. 1999 Feb 2
60 10068510 Mechanism of biotin responsiveness in biotin-responsive multiple carboxylase deficiency. 1999 Feb 4
61 10437643 Prenatal diagnosis of holocarboxylase synthetase deficiency by assay of the enzyme in chorionic villus material followed by prenatal treatment. 1999 Jun 15 1
62 10590022 Relationship between kinetic properties of mutant enzyme and biochemical and clinical responsiveness to biotin in holocarboxylase synthetase deficiency. 1999 Dec 6
63 9630604 Identification of holocarboxylase synthetase (HCS) proteins in human placenta. 1998 Jun 11 2
64 9758715 Deficiency of biotinyl-AMP synthetase activity in fibroblasts of patients with holocarboxylase synthetase deficiency. 1998 Aug 1
65 9128289 Five patients with a biotin-responsive defect in holocarboxylase formation: evaluation of responsiveness to biotin therapy in vivo and comparative biochemical studies in vitro. 1997 May 1
66 9183268 Resolution of subependymal cysts in neonatal holocarboxylase synthetase deficiency. 1997 Apr 2
67 9350481 Multiple carboxylase deficiency: inherited and acquired disorders of biotin metabolism. 1997 2
68 9396568 Characterization of mutant holocarboxylase synthetase (HCS): a Km for biotin was not elevated in a patient with HCS deficiency. 1997 Dec 2
69 8587199 [Cloning of the holocarboxylase synthetase cDNA and identification of mutations prevalent in Japanese HCS-deficient patients]. 1996 Jan 4
70 8814349 Enzymatic diagnosis of holocarboxylase synthetase deficiency using apo-carboxyl carrier protein as a substrate. 1996 Jul 15 1
71 8817339 Clustering of mutations in the biotin-binding region of holocarboxylase synthetase in biotin-responsive multiple carboxylase deficiency. 1996 Jul 3
72 7753853 Isolation of a cDNA encoding human holocarboxylase synthetase by functional complementation of a biotin auxotroph of Escherichia coli. 1995 May 9 5
73 7842009 Isolation and characterization of mutations in the human holocarboxylase synthetase cDNA. 1994 Oct 3
74 3148068 Biotin-responsive multiple carboxylase deficiency in an 8-year-old boy with normal serum biotinidase and fibroblast holocarboxylase-synthetase activities. 1988 1
75 2864473 Enzyme studies in biotin-responsive disorders. 1985 1
76 3920902 Heterogeneity of holocarboxylase synthetase in patients with biotin-responsive multiple carboxylase deficiency. 1985 Mar 3
77 6614920 Regulation and intracellular localization of the biotin holocarboxylase synthetase of 3T3-L1 cells. 1983 Aug 1
78 7102675 Evidence for a defect of holocarboxylase synthetase activity in cultured lymphoblasts from a patient with biotin-responsive multiple carboxylase deficiency. 1982 Jul 1