Published in Proc Natl Acad Sci U S A on January 15, 1991
Tetrahydrobiopterin biosynthesis, regeneration and functions. Biochem J (2000) 3.91
Identity of 4a-carbinolamine dehydratase, a component of the phenylalanine hydroxylation system, and DCoH, a transregulator of homeodomain proteins. Proc Natl Acad Sci U S A (1992) 1.36
Mutation in the 4a-carbinolamine dehydratase gene leads to mild hyperphenylalaninemia with defective cofactor metabolism. Am J Hum Genet (1993) 1.10
Three-dimensional structure of the bifunctional protein PCD/DCoH, a cytoplasmic enzyme interacting with transcription factor HNF1. EMBO J (1995) 1.00
"7-tetrahydrobiopterin," a naturally occurring analogue of tetrahydrobiopterin, is a cofactor for and a potential inhibitor of the aromatic amino acid hydroxylases. Proc Natl Acad Sci U S A (1992) 0.94
Studies on the enzymatic and transcriptional activity of the dimerization cofactor for hepatocyte nuclear factor 1. Proc Natl Acad Sci U S A (1997) 0.93
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). Am J Hum Genet (1998) 0.93
High-resolution structures of the bifunctional enzyme and transcriptional coactivator DCoH and its complex with a product analogue. Protein Sci (1996) 0.93
Identification of hepatic nuclear factor 1 binding sites in the 5' flanking region of the human phenylalanine hydroxylase gene: implication of a dual function of phenylalanine hydroxylase stimulator in the phenylalanine hydroxylation system. Proc Natl Acad Sci U S A (1998) 0.89
Characterization of the wild-type form of 4a-carbinolamine dehydratase and two naturally occurring mutants associated with hyperphenylalaninemia. Proc Natl Acad Sci U S A (1995) 0.83
Overexpression of pterin-4a-carbinolamine dehydratase/dimerization cofactor of hepatocyte nuclear factor 1 in human colon cancer. Am J Pathol (1999) 0.80
HPLC analysis of tetrahydrobiopterin and its pteridine derivatives using sequential electrochemical and fluorimetric detection: application to tetrahydrobiopterin autoxidation and chemical oxidation. Arch Biochem Biophys (2012) 0.79
Enhanced synthesis of 5-hydroxy-l-tryptophan through tetrahydropterin regeneration. AMB Express (2013) 0.75
Characterization of a novel pterin intermediate formed in the catalytic cycle of tyrosine hydroxylase. Biochem J (1996) 0.75
Phenylpyruvic oligophrenia deficiency of phenylalanine-oxidizing system. Proc Soc Exp Biol Med (1953) 2.30
Biosynthesis and metabolism of tetrahydrobiopterin and molybdopterin. Annu Rev Biochem (1985) 2.30
Phenylketonuria due to a deficiency of dihydropteridine reductase. N Engl J Med (1975) 2.18
The isolation and characterization of dihydropteridine reductase from sheep liver. J Biol Chem (1972) 2.02
Purification and some physical properties of phenylalanine hydroxylase from rat liver. J Biol Chem (1970) 1.69
A simple purification of phenylalanine hydroxylase by substrate-induced hydrophobic chromatography. J Biol Chem (1979) 1.68
THE STRUCTURE OF THE PHENYLALANINE-HYDROXYLATION COFACTOR. Proc Natl Acad Sci U S A (1963) 1.57
Phenylalanine hydroxylation cofactor in phenylketonuria. Science (1958) 1.48
A protein that stimulates rat liver phenylalanine hydroxylase. J Biol Chem (1970) 1.41
The enzymatic conversion of phenylalanine to tyrosine. J Biol Chem (1957) 1.31
Letter: Biopterin derivatives in atypical phenylketonuria. Lancet (1976) 1.27
Primapterin, anapterin, and 6-oxo-primapterin, three new 7-substituted pterins identified in a patient with hyperphenylalaninemia. Biochem Biophys Res Commun (1988) 1.23
Purification and characterization of phenylalanine hydroxylase-stimulating protein from rat liver. J Biol Chem (1973) 1.16
GTP cyclohydrolase I deficiency, a new enzyme defect causing hyperphenylalaninemia with neopterin, biopterin, dopamine, and serotonin deficiencies and muscular hypotonia. Eur J Pediatr (1984) 1.14
A new cofactor required for the enzymatic conversion of phenylalanine to tyrosine. J Biol Chem (1958) 1.14
Letter: New forms of phenylketonuria. Lancet (1975) 1.12
Studies on the mechanisms of action of phenylalanine hydroxylase and its protein stimulator. I. Enzyme concentration dependence of the specific activity of phenylalanine hydroxylase due to a nonenzymatic step. J Biol Chem (1973) 1.07
Phenylalanine 4-monooxygenase from rat liver. Methods Enzymol (1987) 1.05
Letter: A variant of phenylketonuria. Lancet (1975) 1.03
Mechanism of oxygen activation by tyrosine hydroxylase. Biochemistry (1987) 1.02
Isolation and characterization of tetrahydropterin oxidation products generated in the tyrosine 3-monooxygenase (tyrosine hydroxylase) reaction. Eur J Biochem (1987) 1.01
Differential diagnosis of tetrahydrobiopterin deficiency. J Inherit Metab Dis (1985) 0.93
7-Substituted pterins. A new class of mammalian pteridines. J Biol Chem (1990) 0.85
Primapterinuria: a new variant of atypical phenylketonuria. J Inherit Metab Dis (1989) 0.84
Incorporation of molecular oxygen into pyrimidine cofactors by phenylalanine hydroxylase. J Biol Chem (1982) 0.82
Atypical phenylketonuria with "dihydrobiopterin synthetase" deficiency: absence of phosphate-eliminating enzyme activity demonstrated in liver. Eur J Pediatr (1985) 0.81
Neonatal hyperphenylalaninaemia presumably caused by a new variant of biopterin synthetase deficiency. Eur J Pediatr (1988) 0.80
Atypical phenylketonuria with normal dihydropteridine reductase activity. N Engl J Med (1976) 0.79
Cleavage of the 5-amino substituent of pyrimidine cofactors by phenylalanine hydroxylase. J Biol Chem (1980) 0.78
Osteoprotegerin ligand is a cytokine that regulates osteoclast differentiation and activation. Cell (1998) 17.76
TRAF6 deficiency results in osteopetrosis and defective interleukin-1, CD40, and LPS signaling. Genes Dev (1999) 6.93
RANK is the intrinsic hematopoietic cell surface receptor that controls osteoclastogenesis and regulation of bone mass and calcium metabolism. Proc Natl Acad Sci U S A (2000) 3.95
Molecular cloning and functional characterization of a novel mammalian sphingosine kinase type 2 isoform. J Biol Chem (2000) 3.71
Solubilization and partial purification of tyrosine hydroxylase from bovine adrenal medulla. J Biol Chem (1971) 2.85
The ligand for osteoprotegerin (OPGL) directly activates mature osteoclasts. J Cell Biol (1999) 2.78
Sphingosine 1-phosphate stimulates cell migration through a G(i)-coupled cell surface receptor. Potential involvement in angiogenesis. J Biol Chem (1999) 2.22
Osteoprotegerin reverses osteoporosis by inhibiting endosteal osteoclasts and prevents vascular calcification by blocking a process resembling osteoclastogenesis. J Exp Med (2000) 2.20
Phenylketonuria due to a deficiency of dihydropteridine reductase. N Engl J Med (1975) 2.18
Role of the sphingosine-1-phosphate receptor EDG-1 in PDGF-induced cell motility. Science (2001) 2.17
3,4-dihydroxyphenylethylamine beta-hydroxylase. Physical properties, copper content, and role of copper in the catalytic acttivity. J Biol Chem (1965) 2.13
Rate acceleration by stereopopulation control: models for enzyme action. Proc Natl Acad Sci U S A (1970) 2.13
Osteoprotegerin ligand modulates murine osteoclast survival in vitro and in vivo. Am J Pathol (2000) 2.12
The isolation and characterization of dihydropteridine reductase from sheep liver. J Biol Chem (1972) 2.02
Budd-Chiari syndrome: etiology, diagnosis and management. Medicine (Baltimore) (1982) 1.92
Vascular endothelial growth factor (VEGF) in breast cancer: comparison of plasma, serum, and tissue VEGF and microvessel density and effects of tamoxifen. Cancer Res (2000) 1.88
Purification and characterization of rat kidney sphingosine kinase. J Biol Chem (1998) 1.76
Hyperphenylalaninemia due to a deficiency of biopterin. A variant form of phenylketonuria. N Engl J Med (1978) 1.71
Clinical hypertension and its interaction with diabetes among American Indians and Alaska Natives. Estimated rates from ambulatory care data. Diabetes Care (1993) 1.70
Purification and some physical properties of phenylalanine hydroxylase from rat liver. J Biol Chem (1970) 1.69
Effects of oral folic acid supplementation on endothelial function in familial hypercholesterolemia. A randomized placebo-controlled trial. Circulation (1999) 1.66
NOD2-expressing bone marrow-derived cells appear to regulate epithelial innate immunity of the transplanted human small intestine. Gut (2007) 1.66
EDG-1 links the PDGF receptor to Src and focal adhesion kinase activation leading to lamellipodia formation and cell migration. FASEB J (2001) 1.55
Partial purification and characterization of tryptophan hydroxylase from rabbit hindbrain. J Biol Chem (1972) 1.53
Sphingosine-1-phosphate: signaling inside and out. FEBS Lett (2000) 1.53
Sphingosine kinase expression regulates apoptosis and caspase activation in PC12 cells. J Neurochem (2001) 1.47
Regulation of nitric oxide synthesis by proinflammatory cytokines in human umbilical vein endothelial cells. Elevations in tetrahydrobiopterin levels enhance endothelial nitric oxide synthase specific activity. J Clin Invest (1994) 1.46
In vitro activation of rat liver phenylalanine hydroxylase by phosphorylation. J Biol Chem (1976) 1.45
Malignant hyperphenylalaninaemia--current status (June 1977). J Inherit Metab Dis (1978) 1.45
A protein that stimulates rat liver phenylalanine hydroxylase. J Biol Chem (1970) 1.41
Endogenous modulators and pharmacological inhibitors of histone deacetylases in cancer therapy. Oncogene (2011) 1.38
Interferon-alpha-2b with VMCP for induction in multiple myeloma: the Israel Myeloma Cooperative Group experience. Isr J Med Sci (1995) 1.38
Functions of a new family of sphingosine-1-phosphate receptors. Biochim Biophys Acta (2000) 1.37
K-Sol corneal preservation. Am J Ophthalmol (1985) 1.36
Identity of 4a-carbinolamine dehydratase, a component of the phenylalanine hydroxylation system, and DCoH, a transregulator of homeodomain proteins. Proc Natl Acad Sci U S A (1992) 1.36
Characterization of the human granulocyte-macrophage colony-stimulating factor receptor. J Biol Chem (1988) 1.35
Exogenous and intracellularly generated sphingosine 1-phosphate can regulate cellular processes by divergent pathways. Biochem Soc Trans (2003) 1.33
The development and function of gamma delta T cells. Immunol Today (1990) 1.33
In vitro model for stretch-induced hypertrophy of skeletal muscle. Science (1979) 1.33
Isolation and expression of rat liver sepiapterin reductase cDNA. Proc Natl Acad Sci U S A (1990) 1.31
Tetrahydrobiopterin, a cofactor for rat cerebellar nitric oxide synthase, does not function as a reactant in the oxygenation of arginine. Proc Natl Acad Sci U S A (1991) 1.30
The stimulation of rat liver phenylalanine hydroxylase by lysolecithin and -chymotrypsin. J Biol Chem (1973) 1.27
The phenylalanine hydroxylating system from mammalian liver. Adv Enzymol Relat Areas Mol Biol (1971) 1.26
Tetrahydrobiopterin, the cofactor for aromatic amino acid hydroxylases, is synthesized by and regulates proliferation of erythroid cells. Proc Natl Acad Sci U S A (1989) 1.25
Bacterial translocation in cirrhotic rats stimulates eNOS-derived NO production and impairs mesenteric vascular contractility. J Clin Invest (1999) 1.24
Enhanced protein synthesis in a cell-free system from hypertrophied skeletal muscle. Science (1967) 1.23
Glucagon stimulation of rat hepatic phenylalanine hydroxylase through phosphorylation in vivo. J Biol Chem (1978) 1.22
Proteolytic modification of the amino-terminal and carboxyl-terminal regions of rat hepatic phenylalanine hydroxylase. J Biol Chem (1986) 1.19
Sphingolipid metabolites: members of a new class of lipid second messengers. J Membr Biol (1995) 1.19
Induction of tissue factor-like activity in monocytes by anti-cardiolipin antibodies. J Immunol (1994) 1.19
Effect of jogging on serum low density lipoprotein cholesterol. Artery (1980) 1.18
Surgical approach to short-bowel syndrome. Experience in a population of 160 patients. Ann Surg (1995) 1.17
Chronic, nonocclusive, and maintenance-free central venous cannula in the rat. Am J Physiol (1980) 1.17
Glycolysis in chick embryo cell cultures transformed by Rous sarcoma virus. Cancer Res (1968) 1.17
Purification and characterization of phenylalanine hydroxylase-stimulating protein from rat liver. J Biol Chem (1973) 1.16
Osteoblast precursor cells are found in CD34+ cells from human bone marrow. Stem Cells (1997) 1.16
Atypical phenylketonuria with normal phenylalanine hydroxylase and dihydropteridine reductase activity in vitro. Pediatrics (1977) 1.16
Sphingosine-1-phosphate phosphohydrolase-1 regulates ER stress-induced autophagy. Cell Death Differ (2010) 1.16
Chondroitin sulfate in a new cornea preservation medium. Am J Ophthalmol (1984) 1.15
Metabolism of the phenylalanine hydroxylation cofactor. J Biol Chem (1967) 1.14
Symptomatology in head and neck cancer: a quantitative review of 385 cases. Am J Public Health (1980) 1.14
Rat liver phenylalanine hydroxylase, an iron enzyme. J Biol Chem (1972) 1.13
Virilizing ovarian tumor in a 2 1/2-year-old girl. J Pediatr (1967) 1.12
Letter: New forms of phenylketonuria. Lancet (1975) 1.12
Sphingosine generation, cytochrome c release, and activation of caspase-7 in doxorubicin-induced apoptosis of MCF7 breast adenocarcinoma cells. Cell Death Differ (2001) 1.12
Sclerosing cholangitis. Anatomical distribution of obstructive lesions. Ann Surg (1984) 1.12
The activation of rat liver phenylalanine hydroxylase by limited proteolysis, lysolecithin, and tocopherol phosphate. Changes in conformation and catalytic properties. J Biol Chem (1984) 1.12
Carcinocythemia (carcinoma cell leukemia). An acute leukemia-like picture due to metastatic carcinoma cells. Am J Med (1976) 1.12
Structure and expression of human dihydropteridine reductase. Proc Natl Acad Sci U S A (1987) 1.11
Stereopopulation control. I. Rate enhancement in the lactonizations of 0-hydroxyhydrocinnamic acids. J Am Chem Soc (1972) 1.11
Alpha 7 beta 1 integrin is a component of the myotendinous junction on skeletal muscle. J Cell Sci (1993) 1.11
Mutation in the 4a-carbinolamine dehydratase gene leads to mild hyperphenylalaninemia with defective cofactor metabolism. Am J Hum Genet (1993) 1.10
Prevalence of diagnosed diabetes among American Indians and Alaska Natives, 1987. Estimates from a national outpatient data base. Diabetes Care (1993) 1.10
Effects of acute hypertrophy on the contractile properties of skeletal muscle. Am J Physiol (1968) 1.09
Sphingosine enhances apoptosis of radiation-resistant prostate cancer cells. Cancer Res (2000) 1.09
E2F-1 blocks terminal differentiation and causes proliferation in transgenic megakaryocytes. Mol Cell Biol (1996) 1.09
Detection of hepatic phenylalanine 4-hydroxylase in classical phenylketonuria. Proc Natl Acad Sci U S A (1973) 1.08
Old age, rehabilitation, and research: a review of the issues. Gerontologist (1988) 1.08
Rat liver phenylalanine hydroxylase. Activation by sulfhydryl modification. J Biol Chem (1981) 1.08
Osteoprotegerin and osteoprotegerin ligand effects on osteoclast formation from human peripheral blood mononuclear cell precursors. J Cell Biochem (1999) 1.07
Mendelian hyperphenylalaninemia. Annu Rev Genet (1988) 1.07
Bovine adrenal tyrosine hydroxylase: purification and properties. J Biol Chem (1977) 1.07
Studies on the mechanisms of action of phenylalanine hydroxylase and its protein stimulator. I. Enzyme concentration dependence of the specific activity of phenylalanine hydroxylase due to a nonenzymatic step. J Biol Chem (1973) 1.07
Spectroscopic investigation of ligand interaction with hepatic phenylalanine hydroxylase: evidence for a conformational change associated with activation. Biochemistry (1984) 1.06
The auto-oxidation of tetrahydrobiopterin. Eur J Biochem (1988) 1.05
Determination of the phosphate content of purified proteins. Anal Biochem (1982) 1.05
Phenylalanine 4-monooxygenase from rat liver. Methods Enzymol (1987) 1.05