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Histidinemia
smpdb.ca/view/SMP0125711DiseaseHistidinemia (Histidine Ammonia-Lyase Deficiency; HAL Deficiency; Histidase Deficiency; HIS Deficiency) is an autosomal recessive disease caused by a mutation in the HAL gene which codes for hisitidine ammonia-lyase. A deficiency in this...
Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Histidine · Ademetionine · Adenosine phosphate · Glutamic acid +9 moreSialuria or French Type Sialuria
smpdb.ca/view/SMP0125712DiseaseSialuria is caused by mutation in the gene encoding uridinediphosphate-N-acetylglucosamine 2-epimerase (UDP-GlcNAc 2-epimerase, which causes an excessive synthesis of sialic acid (N-acetylneuraminic acid, NeuAc). This causes accumulation...
Argininemia
smpdb.ca/view/SMP0125765DiseaseArgininemia is caused by a mutation in the gene ARG, encoding liver arginase, which hydrolyses arginine to urea and ornithine in the last step of the urea cycle. A defect in liver arginase causes accumulation of ammonia in blood; arginin...
Drugs: Pyridoxal phosphate · Pyruvic acid · Arginine · Aspartic acid · Ornithine · L-Glutamine +11 moreHypoacetylaspartia
smpdb.ca/view/SMP0125780DiseaseHypoacetylaspartia, also known as N-acetylaspartate (NAA) deficiency is an extremely rare autosomal recessive inborn error of metabolism (IEM) caused by a mutation in the NAT8L gene. This gene encodes the N-acetylaspartate synthase prote...
Drugs: Pyridoxal phosphate · Arginine · Aspartic acid · L-Glutamine · Adenosine phosphate · Glutamic acid +13 moreTasosartan Action Pathway
smpdb.ca/view/SMP0126013Drug actionTasosartan is a selective angiotensin II (AngII) receptor type 1 (AT1) inhibitor. It is used for the treatment of hypertension and heart failure. This drug causes the blockade of the renin-angiotensin-aldosterone system (RAAS) at the lev...
Butabarbital Action Pathway
smpdb.ca/view/SMP0126119Drug actionButabarbital is a barbiturate drug used as a sedative and hypnotic. Butabarbital, or Butisol, is a fast onset barbiturate with short duration of action compared to other barbiturates. This makes butabarbital a useful drug for treating se...
Drugs: Pyridoxal phosphate · Glutamic acid · ATP · Butabarbital · Calcium · Magnesium cation +3 moreEstazolam Action Pathway
smpdb.ca/view/SMP0126125Drug actionEstazolam is a benzodiazepine used for the short-term management of insomnia. Estazolam, a triazolobenzodiazepine derivative, is an oral hypnotic agent with anticonvulsant, hypnotic, and muscle relaxant properties. It has been shown in s...
Etomidate Action Pathway
smpdb.ca/view/SMP0126127Drug actionEtomidate is a short-acting intravenous anesthetic indicated for the induction of anesthesia and supplementation of subpotent anesthesia during short operative procedures. Imidazole derivative anesthetic and hypnotic with little effect o...
Flunitrazepam Action Pathway
smpdb.ca/view/SMP0126128Drug actionFlunitrazepam is a benzodiazepine used to manage anxiety disorders and insomnia. Flunitrazepam is a benzodiazepine with pharmacologic actions similar to those of diazepam that can cause anterograde amnesia. Some reports indicate that it ...
Drugs: Pyridoxal phosphate · Glutamic acid · ATP · Calcium · Magnesium cation · Flunitrazepam +3 moreGlutethimide Action Pathway
smpdb.ca/view/SMP0126131Drug actionGlutethimide is a hypnotic and sedative. Its use has been largely superseded by other drugs. It can be found under the brand name Doriden and is used to treat insomnia. The hypnotic effect is roughly analogous to that of pentobarbital. I...