Dihydroxyphenylserine DOPS

Figure 5. Anaerobic Decarboxylation of Phenylserine, Dihydroxy phenylalanine (DOPA), and Dihydroxyphenylserine (DOPS) by Supernatant from 20% Homogenate of Dog Kidney in 0.1 M Phosphate Buffer...

Therapy in the deficiencies of TH, AADC and DpR is aimed at correcting the neurotransmitter abnormalities. Bypassing the metabolic block using levodopa/carbidopa together with dopamine agonists has led to improvement in TH deficiency [10]. Monoamine oxidase inhibitors, in conjunction with dopamine agonists and vitamin B6 (cofactor for AADC) ameliorated symptoms in AADC deficiency [3] and dihydroxyphenylserine (DOPS - decarboxylated to form norepinephrine) has corrected the norepinephrine deficiency in DpH deficiency [8]. Currently a therapy for MAO-A deficiency has not been described. 

A few experiments were conducted in barbiturate anesthetized cats and dogs wherein DOPA, phenylserine, p-hydroxyphenylserine (DOPS), and 3,4-dihydroxyphenylserine were injected intravenously tjgU isupply was clamped tern-... 

Fig. 2.2. The effect of oral dihydroxyphenylserine (d,/-DOPS - 4 mg/kg) on plasma and urinary norepinephrine (NE) in an adult D H-deficient patient...

Fig. 2.3. Diagnostic flow-chart in the differentiation of defects of biogenic amine neurotransmitter metabolism. The correct differential diagnosis depends on the pattern of amines and their metabolites in either urine, CSF or plasma. represents increased values, [ represents lowered values. 5HIAA 5-hydroxyindoleacetic acid HVA homovanillic acid 5HT serotonin 30MD 3-0-methyldopa DOPAC dihydroxyphenylacetic acid 3MT 3-methoxytyramine NMN norme-tanephrine VMA vanillylmandelic acid DOPS dihydroxyphenylserine MHPG 3-methoxy-4-hydroxyphenylglycol Y yes N no. Plasma and CSF levels have not been analysed but they probably reflect those seen in urine. For interpretation of quantitative results see pathological values.

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