메뉴 건너뛰기
Orphanet Journal of Rare Diseases
Volumn 3, Issue 1, 2008, Pages
Carglumic acid: An additional therapy in the treatment of organic acidurias with hyperammonemia?
Author keywords

[No Author keywords available]
Indexed keywords

CARGLUMIC ACID; GLUTAMIC ACID DERIVATIVE; METHYLMALONIC ACID; PROPIONIC ACID DERIVATIVE;
EID: 40249084245     PISSN: None     EISSN: 17501172     Source Type: Journal    
DOI: 10.1186/1750-1172-3-2     Document Type: Article
Times cited : (33)
References (7)
  • 1
    • 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 aciduria
    • 500823
    • Inhibition by propionyl-coenzyme A of N-acetylglutamate synthetase in rat liver mitochondria. A possible explanation for hyperammonemia in propionic and methylmalonic aciduria. FX Coudé L Sweetman WL Nyhan, J Clin Invest 1979 64 1544 1551 500823
    • (1979) J Clin Invest , vol.64 , pp. 1544-1551
    • Coudé, F.X.1    Sweetman, L.2    Nyhan, W.L.3
  • 2
    • 0041888299 scopus 로고    scopus 로고
    • N-carbamylglutamate enhances ammonia detoxification in a patient with decompensated methylmalonic aciduria
    • 10.1016/S1096-7192(03)00095-7 12948747
    • N-carbamylglutamate enhances ammonia detoxification in a patient with decompensated methylmalonic aciduria. B Gebhardt S Vlaho D Fischer, Mol Genet Metab 2003 79 303 304 10.1016/S1096-7192(03)00095-7 12948747
    • (2003) Mol Genet Metab , vol.79 , pp. 303-304
    • Gebhardt, B.1    Vlaho, S.2    Fischer, D.3
  • 3
    • 19444367885 scopus 로고    scopus 로고
    • N-Carbamylglutamate protects patients with decompensated propionic aciduria from hyperammonaemia
    • 10.1007/s10545-005-5260-7 15877213
    • N-Carbamylglutamate protects patients with decompensated propionic aciduria from hyperammonaemia. B Gebhardt S Dittrich S Parbel, J Inher Metab Dis 2005 28 241 244 10.1007/s10545-005-5260-7 15877213
    • (2005) J Inher Metab Dis , vol.28 , pp. 241-244
    • Gebhardt, B.1    Dittrich, S.2    Parbel, S.3
  • 4
    • 0030772253 scopus 로고    scopus 로고
    • Neurological dysfunction in methylmalonic aciduria probably related to the inhibitory effect of methylmalonate on brain energy production
    • 10.1023/A:1005359416197 9427143
    • Neurological dysfunction in methylmalonic aciduria probably related to the inhibitory effect of methylmalonate on brain energy production. M Wajner JC Coelho, J Inher Metab Dis 1997 20 761 768 10.1023/A:1005359416197 9427143
    • (1997) J Inher Metab Dis , vol.20 , pp. 761-768
    • Wajner, M.1    Coelho, J.C.2
  • 5
    • 0031879678 scopus 로고    scopus 로고
    • Effects of hyperammonaemia on brain function
    • 10.1023/A:1005393104494 9686341
    • Effects of hyperammonaemia on brain function. RF Butterworth, J Inherit Metab Dis 1998 21 6 20 10.1023/A:1005393104494 9686341
    • (1998) J Inherit Metab Dis , vol.21 , pp. 6-20
    • Butterworth, R.F.1
  • 6
    • 0020062339 scopus 로고
    • Correlation between blood ammonia concentration and organic acid accumulation in isovaleric and propionic aciduria
    • 7054746
    • Correlation between blood ammonia concentration and organic acid accumulation in isovaleric and propionic aciduria. FX Coudé H Ogier G Grimber, Pediatrics 1982 69 115 117 7054746
    • (1982) Pediatrics , vol.69 , pp. 115-117
    • Coudé, F.X.1    Ogier, H.2    Grimber, G.3
  • 7
    • 0025976652 scopus 로고
    • N-acetylglutamate synthetase deficiency: Diagnosis, management and follow-up of a rare disorder of ammonia detoxication
    • 10.1007/BF01955939 2044610
    • N-acetylglutamate synthetase deficiency: diagnosis, management and follow-up of a rare disorder of ammonia detoxication. G Schubiger C Bachmann P Barben, Eur J Pediatr 1991 150 353 356 10.1007/BF01955939 2044610
    • (1991) Eur J Pediatr , vol.150 , pp. 353-356
    • Schubiger, G.1    Bachmann, C.2    Barben, P.3

* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.