Urinary L-lactate excretion is increased in renal Fanconi syndrome

Nephrology Dialysis Transplantation

Volumn 19, Issue 7, 2004, Pages 1767-1773

  Thirumurugan, Arumugavelu ^a   Thewles, Andrew ^b   Gilbert, Rodney D ^c   Hulton, Sally Anne ^a   Milford, David V ^a   Lote, Christopher J ^b   Taylor, C Mark ^a  

^a BIRMINGHAM CHILDREN S HOSPITAL   (United Kingdom)

^b UNIVERSITY OF BIRMINGHAM   (United Kingdom)

^c SOUTHAMPTON GENERAL HOSPITAL   (United Kingdom)

Author keywords

Fanconi syndrome; Lactate co transport; Proximal tubule disorders; Urinary lactate cystinosis

Indexed keywords

COTRANSPORTER; CREATININE; LACTATE DEHYDROGENASE; LACTIC ACID; NICOTINAMIDE ADENINE DINUCLEOTIDE; PYRUVIC ACID; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE;

ADOLESCENT; ADULT; ARTICLE; CHILD; CLINICAL ARTICLE; CONFIDENCE INTERVAL; CONTROLLED STUDY; CREATININE URINE LEVEL; FANCONI RENOTUBULAR SYNDROME; FEMALE; HUMAN; INFANT; KIDNEY DISEASE; KIDNEY PROXIMAL TUBULE; MALE; PHOTOMETRY; PRIORITY JOURNAL; SENSITIVITY AND SPECIFICITY; URINALYSIS; URINARY EXCRETION;

ADOLESCENT; CHILD; CHILD, PRESCHOOL; FANCONI SYNDROME; FEMALE; HUMANS; INFANT; LACTIC ACID; MALE;

EID: 3242733801     PISSN: 09310509     EISSN: None     Source Type: Journal    
DOI: 10.1093/ndt/gfh213     Document Type: Article

References (19)

1. Bergeron M, Gougoux A, Noel J, Parent L. The renal Fanconi syndrome. In: Scriver CR, Beaudet AL, Sly WS, Valle D, eds. The Metabolic and Molecular Bases of Inherited Disease, 8th edn. McGraw Hill, London and New York: 2000; 3: 5024-5038
2. Roth KS, Foreman JW, Segal S. The Fanconi syndrome and mechanisms of tubular transport dysfunction. Kidney Int 1981; 20: 705-716
3. Coor C, Salmon RF, Quigley R, Marver D, Baum M. Role of adenosine triphosphate (ATP) and NaK ATPase in the inhibition of proximal tubule transport with intracellular cystine loading. J Clin Invest 1991; 87: 955-961
4. Munnich A, Rustin P, Rotig A et al. Clinical aspects of mitochondrial disorders. J Inherit Metab Dis 1992; 15: 448-455
5. Hatefi Y. The mitochondrial electron transport and oxidative phosphorylation system. Annu Rev Biochem 1985; 54: 1015-1069
6. Johns DR. Mitochondrial DNA and disease. N Engl J Med 1995; 10: 638-644
7. Niaudet P, Rotig A. The kidney in mitochondrial cytopathies. Kidney Int 1997; 51: 1000-1007
8. Kuwertz-Broking E, Koch HG, Marquardt T et al. Renal Fanconi syndrome: first sign of partial respiratory chain complex IV deficiency. Pediatr Nephrol 2000; 14: 489-495
9. Salmon RF, Baum M. Intracellular cystine loading inhibits transport in the rabbit proximal convoluted tubule. J Clin Invest 1990; 85: 340-344
10. Cetinkaya I, Schlatter E, Hirsch JR, Herter P, Harms E, Kleta R. Inhibition of Na(+)-dependent transporters in cystine-loaded renal cells: electrophysiological studies on the Fanconi syndrome of cystinosis. J Am Soc Nephrol 2002: 13: 2085-2093
11. Cherqui S, Sevin C, Hamard G et al. Intralysosomal cystine accumulation in mice lacking cystinosin, the protein defective in cystinosis. Mol Cell Biol 2002; 22: 7622-7632
12. Jackson JD, Smith FG, Litman NN, Yuile CL, Latta H. The Fanconi syndrome with cystinosis, electron microscopy of the renal biopsy specimens from five patients. Am J Med 1962; 33: 893-910
13. Spear GS, Slusser RJ, Tousimis AJ, Taylor CG, Schulman JD. Cystinosis. An ultrastructural and electron-probe study of the kidney with unusual findings. Arch Path Lab Med 1971; 91: 206-221
14. Craig FN. Renal tubular reabsorption, metabolic utilization and isomeric fractionation of lactic acid in the dog. Am J Physiol 1946; 146: 146-159
15. Dell RB, Winters RW. Lactate gradients in the kidney of the dog. Am J Physiol 1967; 213: 301-307
16. Hervy S, Thomas SR. Inner medullary lactate production and urine-concentrating mechanism: a flat medullary model. Am J Physiol (Renal Physiol) 2003; 284: F65-F81
17. Eladari D, Chambey R, Irinopoulou T et al. Polarized expression of different monocarboxylate transporters in the rat medullary thick limbs of Henle. J Biol Chem 1999; 274: 28 420-28 426
18. Halestrap AP, Price NT. The proton-linked monocarboxylate transporter (MCT) family: structure, function and regulation. Biochem J 1999; 343: 281-299
19. Touati G, Rigal O, Lombes A, Frachon P, Giraud M, Ogier de Baulny H. In vivo functional investigations of lactic acid in patients with respiratory chain disorders. Arch Dis Childh 1997; 76: 16-21