What is the metabolic defect in Primary Oxaluria Type II?
Ans. C. D Glycerate DehydrogenasePrimary Hyperoxaluria Type IThe most common form of Primary hyperoxaluria.It is due to a deficiency of the peroxisomal enzyme alanine glyoxylate amino transferase (expressed only in the liver peroxisomes and requires pyridoxine (vitamin B6) as its cofactor)Protein targeting defect.Primary Hyperoxaluria Type II (GlycericAciduria)Due to a deficiency of D glycerate dehydrogenase (glyoxylate reductase enzyme complex)Secondary HyperoxaluriaPyridoxine deficiency (cofactor for alanine glyoxylate aminotransferase)After ingestion of ethylene glycolHigh doses of vitamin C,After administration of the anesthetic agent methoxyflurane (which oxidizes directly to oxalic acid)In patients with inflammatory bowel disease or extensive resection of the bowel (enteric hyperoxaluria).Nonketotic Hyper Glycinemiaa. Due to a defect in the Glycine Cleavage System
Glycine cleavage system
Alanine glyoxalate amino transferase
D GIycerate dehydrogenase
Excess vitamin C
Proteins and Amino Acids