(Q46901672)

26 December 2017

title

Can the DCoHalpha isozyme compensate in patients with 4a-hydroxy-tetrahydrobiopterin dehydratase/DCoH deficiency? (English)

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26 December 2017

author

Joan M Hevel

1

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26 December 2017

author name string

Jason A Stewart

2

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26 December 2017

Katherine L Gross

3

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26 December 2017

June E Ayling

4

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26 December 2017

publication date

19 January 2006

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26 December 2017

Molecular Genetics and Metabolism

published in

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26 December 2017

volume

88

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26 December 2017

issue

1

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26 December 2017

page(s)

38-46

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26 December 2017

Characterization of a Cofactor That Regulates Dimerization of a Mammalian Homeodomain Protein

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1 reference

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Identity of 4a-carbinolamine dehydratase, a component of the phenylalanine hydroxylation system, and DCoH, a transregulator of homeodomain proteins

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A myosin-like dimerization helix and an extra-large homeodomain are essential elements of the tripartite DNA binding structure of LFB1.

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The bifunctional protein DCoH modulates interactions of the homeodomain transcription factor HNF1 with nucleic acids 1 1Edited by M. Yaniv

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Hepatocyte nuclear factor 1 inactivation results in hepatic dysfunction, phenylketonuria, and renal Fanconi syndrome

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Structural basis of dimerization, coactivator recognition and MODY3 mutations in HNF-1alpha

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Hyperphenylalaninemia with high levels of 7-biopterin is associated with mutations in the PCBD gene encoding the bifunctional protein pterin-4a-carbinolamine dehydratase and transcriptional coactivator (DCoH).

7 January 2021

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Primapterin, anapterin, and 6-oxo-primapterin, three new 7-substituted pterins identified in a patient with hyperphenylalaninemia

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Differential expression of chicken dimerization cofactor of hepatocyte nuclear factor-1 (DcoH) and its novel counterpart, DcoHalpha

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Mirk protein kinase is activated by MKK3 and functions as a transcriptional activator of HNF1alpha

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Hyperphenylalaninemia and impaired glucose tolerance in mice lacking the bifunctional DCoH gene

7 January 2021

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The mechanism of cofactor regeneration during phenylalanine hydroxylation.

7 January 2021

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Activity of the bifunctional protein 4a-hydroxy-tetrahydropterin dehydratase/DCoH during human fetal development: correlation with dihydropteridine reductase activity and tetrahydrobiopterin levels

7 January 2021

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Stereospecificity and catalytic function of histidine residues in 4a-hydroxy-tetrahydropterin dehydratase/DCoH.

7 January 2021

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Catalytic characterization of 4a-hydroxytetrahydropterin dehydratase

7 January 2021

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Hepatocyte nuclear factor 1 and C/EBP are essential for the activity of the human apolipoprotein B gene second-intron enhancer

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Sequences containing the second-intron enhancer are essential for transcription of the human apolipoprotein B gene in the livers of transgenic mice

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Mutations in the pterin-4alpha-carbinolamine dehydratase (PCBD) gene cause a benign form of hyperphenylalaninemia

7 January 2021

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A glycosylated liver-specific transcription factor stimulates transcription of the albumin gene

7 January 2021

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A distal dimerization domain is essential for DNA-binding by the atypical HNF1 homeodomain

7 January 2021

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An encyclopedia of mouse genes

7 January 2021

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Biochemical and structural basis for partially redundant enzymatic and transcriptional functions of DCoH and DCoH2

7 January 2021

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7 January 2021

Identifiers

DOI

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26 December 2017

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26 December 2017