Excess of oxalic acid is excreted because of the deficiency of decarbox­ylase or transaminase.
Therefore excretion of 5-hydroxy indole acetic acid and 5-hydroxy tryptamine (serotonin) in the urine is reduced.
Even though it is not toxic to brain it shows neurological signs like irritability, convulsions, muscular hypertonia, tremors and retarded mental development. This is due to the deficiency of tyrosinase enzyme, which is an aerobic oxidase forming melanin in the melanosomes present in melanocytes of the skin. Reserpine as anti-hypertensive drug releases serotonin and is thus available for monoamine oxidase which converts serotonin to 5-HIAA. It is a rare metabolic disease in which transport of mono amino mono carboxylic acids is defective. It is a metabolic defect related to branched chain amino acids like leucine, isoleucine and valine due to deficient decarboxylase enzyme resulting in the accumulation of these amino acids in the CSF and blood. Lysine condenses with ?-ketoglutarate forming a schiffs base and then by the action of dehydrogenase it is reduced to saccharopine which on further dehydrogenation finally forms adipic acid.
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RBC and liver contain a histidine derivative called ergathionine which is a reducing agent and gives false result for glucose level.
Methionine is oxidized to propionyl- CoA which is converted to succinyl-CoA that enters the TCA cycle.


It forms ?-mercepto pyruvic acid which conjugates with cyanide to form thiocyanate and sulphite to form thiosulphate. Methionine is the precursor for the synthesis of choline, creatine, glutathione, epinephrine and taurine which in turn forms the bile acid taurocholic acid.
Urinary excretion is 20- 30 times more than the normal due to the failure in the re-absorption of cystine.
Due to defect in the enzyme phenylalanine hydroxylase (monooxygenase), phenylalanine accumulates in CSF, blood and in tissues.
Phenylalanine competitively inhibits tyrosine resulting in blond hair, blur iris and fair skin.
Albinism is of three types—in Type-I tyrosinase is absent, in type-II tyrosine cannot be transported to the melanosomes due to lack of permease and in type-Ill tyrosine normally converts to DOPA and quinone and thereafter the enzymes are absent.
The main pathway of tryptophan is formation of anthranillic acid and then glutaryl-CoA and acetyl-CoA.
It is a potent vasoconstrictor, stimulates smooth muscle contraction and is a stimulator of cerebral activity. Accumulation of tryptophan results in formation of indole pyruvic and acetic acid, so amino aciduria is observed. The history of the disease is Nephrocalcinosis and recurrent infection of the urinary tract. Cysteine crystals are deposited in many tissues and organs (particularly in the reticuloendothelial system). The clinical symptoms are occurrence of thrombosis, osteoporosis, dislocated lenses in the eyes and frequently, mental retar­dation. Alternative pathways start, leading to the formation of phenyl pyruvic and phenyl lactic acid. Due to increase in phenylalanine in the cells they are not able to utilize other amino acids.


During carcinoid, 60% of this is converted to serotonin, so there is no formation of niacin.
Melatonin the hormone of the pineal body and the peripheral nerves of humans is synthesized from serotonin. It is not found in the urine of normal persons hence is of diagnostic importance for pregnancy. Imidazole pyruvate is formed and excreted in urine which gives a false FeCl3 test for phenylketonuria. Glutamate plays an important role in the transport of potassium (K+) in the central nervous system (CNS).
Thus brain cells are deprived of essential nutrients for their maturation in the early stages.
In tumours like argent affinomas, due to oxida­tion of serotonin there will be excess formation of 5-hydroxy indole acetic acid (5-HIAA) which is excreted in urine.
Symptoms exhibited are appearance of pellagra, skin rashes with intermittent cerebral ataxia and mental retardation. The smell of maple syrup in urine is attributed to keto acids formed from these branched chain amino acids upon transamination.
It forms gamma amino butyric acid (GABA) upon decarboxylation which regulates the neural activity (depresses its activity).



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