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Arginine and Proline Metabolism
smpdb.ca/view/SMP0000020MetabolicThe arginine and proline metabolism pathway illustrates the biosynthesis and metabolism of several amino acids including arginine, ornithine, proline, citrulline, and glutamate in mammals. In adult mammals, the synthesis of arginine take...
Drugs: Pyridoxal phosphate · Ademetionine · Arginine · Aspartic acid · Ornithine · Adenosine phosphate +21 moreGuanidinoacetate Methyltransferase Deficiency (GAMT Deficiency)
smpdb.ca/view/SMP0000188DiseaseGuanidinoacetate Methyltransferase Deficiency (Creatine-Deficiency-Syndrome) is a rare autosomal recessive disease caused by a mutation in the GAMT gene which codes for guanidinoacetate N-methyltransferase. A deficiency in this enzyme re...
Drugs: Pyridoxal phosphate · Ademetionine · Arginine · Aspartic acid · Ornithine · Adenosine phosphate +21 moreProlinemia Type II
smpdb.ca/view/SMP0000208DiseaseProlinemia Type II is caused by mutation in the pyrroline-5-carboxylate dehydrogenase gene (P5CDH) mitochondrial matrix NAD-dependent dehydrogenase. This dehydrogenase is a catalyst for converting pyrroline-5-carboxylate to glutamate in ...
Drugs: Pyridoxal phosphate · Ademetionine · Arginine · Aspartic acid · Ornithine · Adenosine phosphate +21 moreOrnithine Aminotransferase Deficiency (OAT Deficiency)
smpdb.ca/view/SMP0000363DiseaseOrnithine aminotransferase deficiency (OAT deficiency, ornithine keto acid aminotransferase deficiency, OKT deficiency, ornithine-delta-aminotransferase deficiency, gyrate atrophy of the choroid and retina) is an autosomal recessive diso...
Drugs: Pyridoxal phosphate · Ademetionine · Arginine · Aspartic acid · Ornithine · Adenosine phosphate +21 moreHyperornithinemia-Hyperammonemia-Homocitrullinuria (HHH-syndrome)
smpdb.ca/view/SMP0000506DiseaseHyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome is an autosomal recessive disorder of the urea cycle caused by a defective SLC25A15 gene. SLC25A15 codes for the mitochondrial ornithine carrier (ORC1) which transports or...
Drugs: Pyridoxal phosphate · Ademetionine · Arginine · Aspartic acid · Ornithine · Adenosine phosphate +21 moreNitric Oxide Signaling Pathway
smpdb.ca/view/SMP0063777SignalingNitric oxide (NO) is a gaseous chemical transmitter that is produced from the amino acid L-arginine by the members of the NO synthase (NOS) family of proteins. The NOS family of enzymes catalyzes the oxidation of L-arginine to form L-cit...
Isosorbide Mononitrate Action Pathway
smpdb.ca/view/SMP0125275Drug actionIsosorbide mononitrate is an organic nitrate and an antianginal drug used to treat Angina Pectoris through vasodilation. It is an active metabolite of isosorbide dinitrate. As a organic nitrate, it is a prodrug for nitric oxide, which is...
Viral Sepsis
smpdb.ca/view/SMP0125490DiseaseViral sepsis begins when viral coat proteins activate the Toll-like receptors TLR4 and TLR2 on the membranes of macrophages, T-cells and dendritic cells. In addition to this protein activation, the viral DNA (or RNA if it is an RNA virus...
Hyperprolinemia Type II
smpdb.ca/view/SMP0125624DiseaseHyperprolinemia Type II (HPII), also known as 1-pyrroline-5-carboxylate dehydrogenase deficiency, is an extremely rare inborn error of metabolism (IEM) and autosomal recessive disorder of the arginine and proline metabolism pathway. It i...
Drugs: Pyridoxal phosphate · Ademetionine · Arginine · Aspartic acid · Ornithine · Adenosine phosphate +21 moreHyperornithinemia with Gyrate Atrophy (HOGA)
smpdb.ca/view/SMP0125630DiseaseHyperornithinemia with gyrate atrophy (HOGA), also known as gyrate atrophy of the choroid and retina, OAT deficiency, ornithine aminotransferase deficiency, ornithine keto acid aminotransferase deficiency) is an autosomal recessive disor...
Drugs: Pyridoxal phosphate · Ademetionine · Arginine · Aspartic acid · Ornithine · Adenosine phosphate +21 more