EC Number |
Cofactor |
Reference |
---|
4.1.1.28 | more |
no absolute requirement for exogenous pyridoxal 5'-phosphate |
650450 |
4.1.1.28 | pyridoxal 5'-phosphate |
- |
649601, 649696, 650410, 650436, 650868, 653346, 653352, 653356, 653443, 665420, 666154, 690973, 691469, 691846, 691888, 691987, 692200, 692593, 693382, 694362, 701921, 702954, 702968, 703136, 703575, 704020, 704084, 704491, 704803, 705269, 705914, 715979, 716172, 716467, 716702, 743976, 743977, 744008, 744251, 744252, 745291, 748845 |
4.1.1.28 | pyridoxal 5'-phosphate |
(vitamin B6), patients with AAAD deficiency receives a therapeutic trial with pyridoxal-5'-phosphate, individual dosage varies between 150 and 4800 mg/day (20-160 mg/kg per day). 4 of 9 patients improved slightly on vitamin B6 (3 of them are siblings with a special L-dopa responsive mutation). There is no change in CSF neurotransmitter findings in all investigated patients. Side effects are predominantly gastrointestinal, such as nausea, vomiting and abdominal pain. |
693490 |
4.1.1.28 | pyridoxal 5'-phosphate |
0.001-0.1 mM, stimulates 2fold to 3fold |
4232 |
4.1.1.28 | pyridoxal 5'-phosphate |
0.0032-0.32 mM, enhances activity greater than 2fold |
4212 |
4.1.1.28 | pyridoxal 5'-phosphate |
1 mol of pyridoxal 5'-phosphate is bound to the epsilon-amino group of a lysyl residue of the apoenzyme |
4218 |
4.1.1.28 | pyridoxal 5'-phosphate |
5-hydroxytryptophan can be decarboxylated without exogenous addition of pyridoxal 5'-phosphate, addition of pyridoxal 5'-phosphate substantially increases enzyme activity. Excess of coenzyme, above 0.06 mM, induces inhibition in adrenals and liver but not in the central nervous system |
4215 |
4.1.1.28 | pyridoxal 5'-phosphate |
addition of pyridoxal 5'-phosphate increases activity towards 3,4-dihydroxyphenylalanine, o-Tyr, m-Tyr, but not towards 5-hydroxytryptophan |
4213 |
4.1.1.28 | pyridoxal 5'-phosphate |
binding site is located at the amino acid position 313-334 |
726645 |
4.1.1.28 | pyridoxal 5'-phosphate |
cofactor |
4202 |