Names & Taxonomy

Uniprot ID:
P61457
Entry Name:
PHS_HUMAN
Status:
reviewed
Protein Names:
Pterin-4-alpha-carbinolamine dehydratase (PHS) (EC 4.2.1.96) (4-alpha-hydroxy-tetrahydropterin dehydratase) (Dimerization cofactor of hepatocyte nuclear factor 1-alpha) (DCoH) (Dimerization cofactor of HNF1) (Phenylalanine hydroxylase-stimulating protein) (Pterin carbinolamine dehydratase) (PCD)
Gene Names:
PCBD1 DCOH PCBD
Gene Names Primary:
PCBD1
Organism:
Homo sapiens (Human)

Structure

Length:
104
Sequence:
MAGKAHRLSAEERDQLLPNLRAVGWNELEGRDAIFKQFHFKDFNRAFGFMTRVALQAEKLDHHPEWFNVYNKVHITLSTHECAGLSERDINLASFIEQVAVSMT
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Cytoplasm. Nucleus. Note=Cytoplasmic and/or nuclear.

Function

Function:
Involved in tetrahydrobiopterin biosynthesis. Seems to both prevent the formation of 7-pterins and accelerate the formation of quinonoid-BH2. Coactivator for HNF1A-dependent transcription. Regulates the dimerization of homeodomain protein HNF1A and enhances its transcriptional activity.
Catalytic Activity:
(6R)-6-(L-erythro-1,2-dihydroxypropyl)-5,6,7,8-tetrahydro-4a-hydroxypterin = (6R)-6-(L-erythro-1,2-dihydroxypropyl)-7,8-dihydro-6H-pterin + H(2)O.
Gene Ontology Go:
cytoplasm
cytosol
extracellular exosome
nucleoplasm
4-alpha-hydroxytetrahydrobiopterin dehydratase activity
identical protein binding
phenylalanine 4-monooxygenase activity
transcription coactivator activity
cellular nitrogen compound metabolic process
L-phenylalanine catabolic process
positive regulation of transcription, DNA-templated
protein heterooligomerization
protein homotetramerization
regulation of protein homodimerization activity
small molecule metabolic process
tetrahydrobiopterin biosynthetic process
transcription, DNA-templated
Gene Ontology Biological Process:
cellular nitrogen compound metabolic process
L-phenylalanine catabolic process
positive regulation of transcription, DNA-templated
protein heterooligomerization
protein homotetramerization
regulation of protein homodimerization activity
small molecule metabolic process
tetrahydrobiopterin biosynthetic process
transcription, DNA-templated
Gene Ontology Molecular Function:
4-alpha-hydroxytetrahydrobiopterin dehydratase activity
identical protein binding
phenylalanine 4-monooxygenase activity
transcription coactivator activity
Gene Ontology Cellular Component:
cytoplasm
cytosol
extracellular exosome
nucleoplasm
Keywords:
Acetylation
Activator
Complete proteome
Cytoplasm
Direct protein sequencing
Disease mutation
Lyase
Nucleus
Reference proteome
Tetrahydrobiopterin biosynthesis
Transcription
Transcription regulation
Interacts With:
Itself; Q9UKV3; P05067; P51116; Q8TBB1; Q07654

Publication

PubMed ID:
1763325 7763270 10098606 15489334 8444860 21269460 24275569 8352282 9760199