Hydroxyproline metabolism in mouse models of primary hyperoxaluria
نویسندگان
چکیده
John Knight, Ross P. Holmes, Scott D. Cramer, Tatsuya Takayama, and Eduardo Salido Departments of Urology and Cancer Biology, Wake Forest University Health Sciences, Winston-Salem, North Carolina; Department of Pharmacology, University of Colorado, Denver, Anschutz Medical Campus, Aurora, Colorado; and Center for Biomedical Research on Rare Diseases, Hospital Universitario Canarias, Instituto Tecnologías Biomédicas, University of La Laguna, La Laguna, Spain
منابع مشابه
Re: hydroxyproline metabolism in mouse models of primary hyperoxaluria.
Primary hyperoxaluria type 1 (PH1) and type 2 (PH2) are rare genetic diseases that result from deficiencies in glyoxylate metabolism. The increased oxalate synthesis that occurs can lead to kidney stone formation, deposition of calcium oxalate in the kidney and other tissues, and renal failure. Hydroxyproline (Hyp) catabolism, which occurs mainly in the liver and kidney, is a prominent source o...
متن کاملThe enzyme 4-hydroxy-2-oxoglutarate aldolase is deficient in primary hyperoxaluria type III
During the last years, the group of patients with the typical clinical signs of primary hyperoxaluria (PH), but negative diagnostic results for the two types of PH known up till then has grown increasingly larger [1, 2]. It was, however, always obvious that the dramatic clinical course of most of these patients with unclassified hyperoxaluria, e.g. recurrent calcium-oxalate (CaOx) kidney stones...
متن کاملThe Effect of Vitamin B6 Deficiency in Rats on the Metabolism of Oxalic Acid Precursors.
Studies from this laboratory in rats, cats, and monkeys’ have shown that vitamin B6 deficiency is accompanied by a marked increase in endogenous urinary oxalate excretion (1, 2). Studies of oxalate formation are of interest because of the importance of oxalate in urinary calculi (3) and in the genetic disorder, primary hyperoxaluria (4). In this paper alterations caused by vitamin Bg deficiency...
متن کاملHyperoxalurias and their treatment
Hyperoxaluria is characterized by an increased in excretion of oxalate by kidney.There are two distinct clinical expressions of hyperoxaluria, named primary and secondary hyperoxaluria. Primary hyperoxaluria is a genetic disorder due to defective enzyme activity .In contrast , secondary hyperoxaluria , is caused by increased dietary ingestion of oxalate or oxalate precursors. There are three ma...
متن کاملStructural and Biochemical Studies of Human 4-hydroxy-2-oxoglutarate Aldolase: Implications for Hydroxyproline Metabolism in Primary Hyperoxaluria
BACKGROUND 4-hydroxy-2-oxoglutarate (HOG) aldolase is a unique enzyme in the hydroxyproline degradation pathway catalyzing the cleavage of HOG to pyruvate and glyoxylate. Mutations in this enzyme are believed to be associated with the excessive production of oxalate in primary hyperoxaluria type 3 (PH3), although no experimental data is available to support this hypothesis. Moreover, the identi...
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تاریخ انتشار 2012