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Volumn 145, Issue 4, 2004, Pages 552-554

Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate

Author keywords

[No Author keywords available]

Indexed keywords

BENZOIC ACID; CARGLUMIC ACID; CITRULLINE; GLUTAMATE ACETYLTRANSFERASE; N CARBAMYLGLUTAMATE; PEPTIDE DERIVATIVE; UNCLASSIFIED DRUG;

EID: 5144230046     PISSN: 00223476     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.jpeds.2004.06.047     Document Type: Article
Times cited : (58)

References (11)
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    • N-acetylglutamate and its changing role through evolution
    • L. Caldovic, and M. Tuchman N-acetylglutamate and its changing role through evolution Biochem J 372 2003 279 290
    • (2003) Biochem J , vol.372 , pp. 279-290
    • Caldovic, L.1    Tuchman, M.2
  • 2
    • 0020392133 scopus 로고
    • N-acetylglutamate synthetase (NAGS) deficiency: Diagnosis, clinical observations and treatment
    • C. Bachmann, J.P. Colombo, and K. Jaggi N-acetylglutamate synthetase (NAGS) deficiency: diagnosis, clinical observations and treatment Adv Exp Med Biol 153 1982 39 45
    • (1982) Adv Exp Med Biol , vol.153 , pp. 39-45
    • Bachmann, C.1    Colombo, J.P.2    Jaggi, K.3
  • 5
    • 0038485942 scopus 로고    scopus 로고
    • Null mutations in the N-acetylglutamate synthase gene associated with acute neonatal disease and hyperammonemia
    • L. Caldovic, H. Morizono, M. Panglao, S.F. Cheng, S. Packman, and M. Tuchman Null mutations in the N-acetylglutamate synthase gene associated with acute neonatal disease and hyperammonemia Hum Genet 112 2003 364 368
    • (2003) Hum Genet , vol.112 , pp. 364-368
    • Caldovic, L.1    Morizono, H.2    Panglao, M.3    Cheng, S.F.4    Packman, S.5    Tuchman, M.6
  • 8
    • 0015909861 scopus 로고
    • Vitamin-responsive inborn errors of metabolism
    • C.R. Scriver Vitamin-responsive inborn errors of metabolism Metabolism 22 1973 1319 1344
    • (1973) Metabolism , vol.22 , pp. 1319-1344
    • Scriver, C.R.1
  • 9
    • 0020612790 scopus 로고
    • Inhibition of ureagenesis by valproate in rat hepatocytes. Role of N- acetylglutamate and acetyl-CoA
    • F.X. Coude, G. Grimber, P. Parvy, D. Rabier, and F. Petit Inhibition of ureagenesis by valproate in rat hepatocytes. Role of N- acetylglutamate and acetyl-CoA Biochem J 216 1983 233 236
    • (1983) Biochem J , vol.216 , pp. 233-236
    • Coude, F.X.1    Grimber, G.2    Parvy, P.3    Rabier, D.4    Petit, F.5
  • 10
    • 0018595957 scopus 로고
    • Inhibition by propionyl-coenzyme a of N-acetylglutamate synthetase in rat liver mitochondria. A possible explanation for hyperammonemia in propionic and methylmalonic acidemia
    • F.X. Coude, L. Sweetman, and W.L. Nyhan Inhibition by propionyl-coenzyme A of N-acetylglutamate synthetase in rat liver mitochondria. A possible explanation for hyperammonemia in propionic and methylmalonic acidemia J Clin Invest 64 1979 1544 1551
    • (1979) J Clin Invest , vol.64 , pp. 1544-1551
    • Coude, F.X.1    Sweetman, L.2    Nyhan, W.L.3
  • 11
    • 0041888299 scopus 로고    scopus 로고
    • N-carbamylglutamate enhances ammonia detoxification in a patient with decompensated methylmalonic aciduria
    • B. Gebhardt, S. Vlaho, D. Fischer, A. Sewell, and H. Bohles N-carbamylglutamate enhances ammonia detoxification in a patient with decompensated methylmalonic aciduria Mol Genet Metabol 79 2003 303 304
    • (2003) Mol Genet Metabol , vol.79 , pp. 303-304
    • Gebhardt, B.1    Vlaho, S.2    Fischer, D.3    Sewell, A.4    Bohles, H.5


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.