Oral supplementation corrects plasma lysine concentrations in lysinuric protein intolerance

Metabolism: Clinical and Experimental

Volumn 52, Issue 7, 2003, Pages 935-938

  Lukkarinen, Mari ^a,b   Näntö Salonen, Kirsti ^a,b   Pulkki, Kari ^a,b   Aalto, Maija ^a,b   Simell, Olli ^a,b  

^a UNIVERSITY OF TURKU   (Finland)

^b TURKU UNIVERSITY HOSPITAL   (Finland)

Author keywords

[No Author keywords available]

Indexed keywords

ARGININE; CITRULLINE; LYSINE; ORNITHINE; PROTEIN;

ABDOMINAL CRAMP; ADULT; ARTICLE; CLINICAL ARTICLE; CONTROLLED STUDY; DIARRHEA; DIET SUPPLEMENTATION; DRUG DOSE REGIMEN; DRUG SAFETY; DRUG TOLERABILITY; FEMALE; GASTROINTESTINAL SYMPTOM; HUMAN; LOW DRUG DOSE; LYSINURIC PROTEIN INTOLERANCE; MALE; MEAL; PRIORITY JOURNAL; PROTEIN BLOOD LEVEL; PROTEIN INTOLERANCE; SHORT COURSE THERAPY; VERNER MORRISON SYNDROME;

EID: 0042591428     PISSN: 00260495     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0026-0495(03)00089-1     Document Type: Article

References (18)

1. Torrents D, Mykkanen J, Pineda M, et al: Identification of SLC7A7, encoding y+LAT-1, as the lysinuric protein intolerance gene. Nat Genet 21:293-296,1999
2. Rajantie J, Rapola J, Siimes MA: Ferritinemia with subnormal iron stores in Lysinuric protein intolerance. Metabolism 30:3-5, 1981
3. Rajantie J, Perheentupa J: Lysinuric protein intolerance. Lancet 2:978, 1980 (letter)
4. Smith DW, Scriver CR, Tenenhouse HS, et al: Lysinuric protein intolerance mutation is expressed in the plasma membrane of cultured skin fibroblasts. Proc Natl Acad Sci USA 84:7711-7715, 1987
5. Simell O, Perheentupa J, Rapola J, et al: Lysinuric protein intolerance. Am J Med 59:229-240, 1975
6. Lukkarinen M, Nanto-Salonen K, Ruuskanen O, et al: Varicella and varicella immunity in patients with lysinuric protein intolerance. J Inherit Metab Dis 21:103-111, 1998
7. Rajantie J, Simell O, Rapola J, et al: Lysinuric protein intolerance: A two-year trial of dietary supplementation therapy with citrulline and lysine. J Pediatr 97:927-932, 1980
8. Awrich AE, Stackhouse WJ, Cantrell JE, et al: Hyperdibasicaminoaciduria: Hyperammonemia, and growth retardation: Treatment with arginine, lysine, and citrulline. J Pediatr 87:731-738, 1975
9. Svedstron E, Parto K, Marttinen M, et al: Skeletal manifestation of lysinuric protein intolerance. A follow-up study of 29 patients. Skeletal Radiol 22:11-16, 1993
10. Parto K, Penttinen R, Paronen I, et al: Osteoporosis in lysinuric protein intolerance. J Inherit Metab Dis 16:441-450, 1993
11. Civitelli R, Villareal DT, Agnusdei D, et al: Dietary L-lysine and calcium metabolism in humans. Nutrition 8:400-405, 1992
12. Flodin NW: The metabolic roles, pharmacology, and toxicology of lysine. J Am Coll Nutr 16:7-21, 1997
13. Omura K, Yamanaka N, Higami S, et al: Lysine malabsorption syndrome: A new type of transport defect. Pediatrics 57:102-105, 1976
14. Kato T, Sano M, Mizutani N: Inhibitory effect of intravenous lysine infusion on urea cycle metabolism. Eur J Pediatr 56-58, 1987
15. Parvey P, Bardet J, Rabier D, et al: A scheme for the interpretation of primary and secondary disturbances of plasma and urinary amino acid profiles. A possible way to expert system. Clin Chim Acta 235:1-10, 1995
16. Candito M, Vianey Saban C, Ferraci JP, et al: Lysinuric protein intolerance. Urinary amino acid excretion at 2 and 9 days of age. J Inherit Metab Dis 17:252-253, 1994
17. De Vrese M, Barth CA: Influence of lysine on urea cycle activity and orotate formation in the isolated perfused rat liver. Biol Chem Hoppe Seyler 366:455-461, 1985
18. Lukkarinen M, Nanto-Salonen K, Pulkki K, et al: Effect of lysine infusion on urea cycle in lysinuric protein intolerance. Metabolism 49:621-625, 2000