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Identification
HMDB Protein ID HMDBP10140
Secondary Accession Numbers
  • 16096
Name Pterin-4-alpha-carbinolamine dehydratase 2
Synonyms
  1. 4-alpha-hydroxy-tetrahydropterin dehydratase 2
  2. DcoH-like protein DCoHm
  3. Dimerization cofactor of hepatocyte nuclear factor 1 from muscle
  4. HNF-1-alpha dimerization cofactor
  5. PHS 2
Gene Name PCBD2
Protein Type Enzyme
Biological Properties
General Function Involved in 4-alpha-hydroxytetrahydrobiopterin dehydratase activity
Specific Function Involved in tetrahydrobiopterin biosynthesis. Seems to both prevent the formation of 7-pterins and accelerate the formation of quinonoid-BH2 (By similarity). Regulates the dimerization of homeodomain protein HNF-1-alpha and enhances its transcriptional activity.
Pathways Not Available
Reactions
(6R)-6-(L-erythro-1,2-dihydroxypropyl)-5,6,7,8-tetrahydro-4a-hydroxypterin → Dihydrobiopterin + Water details
4a-Hydroxytetrahydrobiopterin → 4a-Carbinolamine tetrahydrobiopterin + Water details
GO Classification
Biological Process
positive regulation of transcription, DNA-dependent
protein heterooligomerization
protein homotetramerization
tetrahydrobiopterin biosynthetic process
Cellular Component
mitochondrion
nucleus
Function
catalytic activity
lyase activity
4-alpha-hydroxytetrahydrobiopterin dehydratase activity
carbon-oxygen lyase activity
hydro-lyase activity
Molecular Function
phenylalanine 4-monooxygenase activity
4-alpha-hydroxytetrahydrobiopterin dehydratase activity
Process
metabolic process
biosynthetic process
cellular biosynthetic process
heterocycle biosynthetic process
pteridine and derivative biosynthetic process
tetrahydrobiopterin biosynthetic process
Cellular Location Not Available
Gene Properties
Chromosome Location 5
Locus 5q31.1
SNPs PCBD2
Gene Sequence
>393 bp
ATGGCGGCGGTGCTCGGGGCGCTCGGGGCGACGCGGCGCTTGTTGGCGGCGCTGCGAGGC
CAGAGCCTAGGGCTAGCGGCCATGTCATCAGGTACTCACAGGTTGACTGCAGAGGAGAGG
AACCAAGCTATACTTGACCTTAAAGCAGCAGGATGGTCGGAATTAAGTGAGAGAGATGCC
ATCTACAAAGAATTCTCCTTCCACAATTTTAATCAGGCATTTGGCTTTATGTCCCGAGTT
GCCCTACAAGCAGAGAAGATGAATCATCACCCAGAATGGTTCAATGTATACAACAAGGTC
CAGATAACTCTCACCTCACATGACTGTGGTGAACTGACCAAAAAAGATGTGAAGCTGGCC
AAGTTTATTGAAAAAGCAGCTGCTTCTGTGTGA
Protein Properties
Number of Residues 130
Molecular Weight 14365.325
Theoretical pI 9.115
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Pterin-4-alpha-carbinolamine dehydratase 2
MAAVLGALGATRRLLAALRGQSLGLAAMSSGTHRLTAEERNQAILDLKAAGWSELSERDA
IYKEFSFHNFNQAFGFMSRVALQAEKMNHHPEWFNVYNKVQITLTSHDCGELTKKDVKLA
KFIEKAAASV
GenBank ID Protein 134133240
UniProtKB/Swiss-Prot ID Q9H0N5
UniProtKB/Swiss-Prot Entry Name PHS2_HUMAN
PDB IDs Not Available
GenBank Gene ID NM_032151.4
GeneCard ID PCBD2
GenAtlas ID PCBD2
HGNC ID HGNC:24474
References
General References
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  2. Choudhary C, Kumar C, Gnad F, Nielsen ML, Rehman M, Walther TC, Olsen JV, Mann M: Lysine acetylation targets protein complexes and co-regulates major cellular functions. Science. 2009 Aug 14;325(5942):834-40. doi: 10.1126/science.1175371. Epub 2009 Jul 16. [PubMed:19608861 ]
  3. Wiemann S, Weil B, Wellenreuther R, Gassenhuber J, Glassl S, Ansorge W, Bocher M, Blocker H, Bauersachs S, Blum H, Lauber J, Dusterhoft A, Beyer A, Kohrer K, Strack N, Mewes HW, Ottenwalder B, Obermaier B, Tampe J, Heubner D, Wambutt R, Korn B, Klein M, Poustka A: Toward a catalog of human genes and proteins: sequencing and analysis of 500 novel complete protein coding human cDNAs. Genome Res. 2001 Mar;11(3):422-35. [PubMed:11230166 ]
  4. Schmutz J, Martin J, Terry A, Couronne O, Grimwood J, Lowry S, Gordon LA, Scott D, Xie G, Huang W, Hellsten U, Tran-Gyamfi M, She X, Prabhakar S, Aerts A, Altherr M, Bajorek E, Black S, Branscomb E, Caoile C, Challacombe JF, Chan YM, Denys M, Detter JC, Escobar J, Flowers D, Fotopulos D, Glavina T, Gomez M, Gonzales E, Goodstein D, Grigoriev I, Groza M, Hammon N, Hawkins T, Haydu L, Israni S, Jett J, Kadner K, Kimball H, Kobayashi A, Lopez F, Lou Y, Martinez D, Medina C, Morgan J, Nandkeshwar R, Noonan JP, Pitluck S, Pollard M, Predki P, Priest J, Ramirez L, Retterer J, Rodriguez A, Rogers S, Salamov A, Salazar A, Thayer N, Tice H, Tsai M, Ustaszewska A, Vo N, Wheeler J, Wu K, Yang J, Dickson M, Cheng JF, Eichler EE, Olsen A, Pennacchio LA, Rokhsar DS, Richardson P, Lucas SM, Myers RM, Rubin EM: The DNA sequence and comparative analysis of human chromosome 5. Nature. 2004 Sep 16;431(7006):268-74. [PubMed:15372022 ]
  5. Lim S, Jin K, Friedman E: Mirk protein kinase is activated by MKK3 and functions as a transcriptional activator of HNF1alpha. J Biol Chem. 2002 Jul 12;277(28):25040-6. Epub 2002 Apr 29. [PubMed:11980910 ]