PMID: 9540857Apr 16, 1998Paper

Secondary abnormality of carnitine biosynthesis results from carnitine reabsorptional system defect in juvenile visceral steatosis mice

Biochimica Et Biophysica Acta
M HoriuchiTakeyori Saheki

Abstract

We characterized the L-carnitine transport system which is defective in the kidney of juvenile visceral steatosis (JVS) mice by using kidney slices and carnitine-related compounds, and evaluated the influence of the transport defect on the biosynthetic pathway of carnitine. The JVS mouse transport system defect, calculated as the difference in the transport activity between control and JVS mice, was simulated in control by gamma-butyrobetaine (gamma-BB) and acetyl L-carnitine. gamma-BB hydroxylase activity in the liver of JVS mice was double that of control mice, but the hepatic level of gamma-BB in JVS mice was lower than in control mice, suggesting that the conversion of gamma-BB to carnitine is not activated in the liver of JVS mice. JVS mice showed higher fractional excretions not only of L-carnitine but also of gamma-BB and acetyl L-carnitine than control mice, indicating disturbed reabsorption of gamma-BB and acetyl L-carnitine. The disturbed reabsorption of gamma-BB in JVS mice is consistent with the fact that the amount of urinary gamma-BB in JVS mice was four times that of control. The sum of the concentrations of L-carnitine, acetyl L-carnitine and gamma-BB in the urine of JVS mice was not significantly different from...Continue Reading

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Citations

Aug 15, 2002·Molecular Genetics and Metabolism·Karim LahjoujiIjaz A Qureshi
Jan 23, 2002·The Biochemical Journal·Frédéric M Vaz, Ronald J A Wanders
May 20, 2014·Pharmacological Research : the Official Journal of the Italian Pharmacological Society·Edgars LiepinshMaija Dambrova
May 9, 2001·The Journal of Pharmacy and Pharmacology·Y HigashiA Tsuji
Aug 18, 2001·Molecular Genetics and Metabolism·K LahjoujiI A Qureshi

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