EC Number |
General Information |
Reference |
---|
4.2.1.96 | malfunction |
a Mu transposon insertion in the Zea mays (maize) gene encoding a chloroplast dimerization co-factor of hepatocyte nuclear factor 1 (DCoH)/pterin-4a-carbinolamine dehydratases (PCD)-like protein is the causative mutation in a seedling-lethal, Rubisco-deficient mutant named Rubisco accumulation factor 2 (raf2-1). In raf2 mutants newly synthesized Rubisco large subunit accumulates in a high-molecular weight complex, the formation of which requires a specific chaperonin 60-kDa isoform. raf2 Mutant phenotype, overview |
748945 |
4.2.1.96 | malfunction |
in mutant phenotype lemon, the expression of BmDhpr is activated in the brain and sexual glands while BmPcd is expressed in a wider special pattern when the de novo pathway of BH4 is missing |
742313 |
4.2.1.96 | malfunction |
PCD deficiency causes in newborns a mild form of hyperphenylalaninaemia with persistent high urinary levels of primapterin. Affected patients appear completely normal, but have elevated phenylalanine levels at birth, which normalize after few months of life and remain normal or just above the normal range of phenylalanine with an unrestricted diet |
704967 |
4.2.1.96 | metabolism |
pterin-4a-carbinolamine dehydratase (PCD, BmPcd) and dihydropteridine reductase (DHPR, BmDhpr) might normally act in the regeneration pathway of Bombyx mori |
742313 |
4.2.1.96 | metabolism |
the PCD activity of the homoteterameric enzyme is involved in the aromatic amino acid metabolism |
714176 |
4.2.1.96 | physiological function |
expression of phenylalanine hydroxylase, PhhA (whereby folX and folM are essential for its function), plus the recycling enzyme pterin 4a-carbinolamine dehydratase, PhhB, rescue tyrosine auxotrophy in Escherichia coli |
704330 |
4.2.1.96 | physiological function |
PCD is required for the regeneration of tetrahydrobiopterin after phenylalanine hydroxylation. PCD can dimerize with HNF-1a and work as a transcription factor |
704967 |
4.2.1.96 | physiological function |
pterin-4a-carbinolamine dehydratase regenerates the tetrahydropterin-dependent aromatic amino acid hydroxylase, AAH, tetrahydropterin cofactor from 4a-carbinolamine |
716517 |
4.2.1.96 | physiological function |
the bifunctional protein shows two disparate functions, i.e. dimerization cofactor of HNF-1, DCoH1, and pterin-4a-carbinolamine dehydratase, PCD, that are associated with a change in oligomeric state between dimer and tetramer, overview. The PCD activity of homotetramers aids in aromatic amino acidmetabolism |
714176 |
4.2.1.96 | physiological function |
the maize gene Rubisco accumulation factor 2 (raf2) is required for Rubisco biogenesis. RAF2 is conserved among photosynthetic eukaryotes and is related to a mitochondrial protein: dimerization co-factor of hepatocyte nuclear factor 1 (DCoH)/pterin-4a-carbinolamine dehydratases (PCD)-like protein. The pterin-4a-carbinolamine dehydratases (PCD)-like protein RAF2 lacks PCD activity acquires a chloroplast-specific domain. Effect of RAF2 on the accumulation of rbcL and rbcs transcripts is investigated by gel blot analysis of total seedling leaf RNA from wild-type and raf2 mutants |
748945 |