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Volumn 103, Issue 4, 2011, Pages 399-400

Blood phenylalanine clearance and BH 4-responsiveness in classic phenylketonuria

Author keywords

Dietary treatment; Inborn error of metabolism; PAH; Phenylalanine hydroxylase; Phenylalanine kinetics; PKU

Indexed keywords

PHENYLALANINE; PHENYLALANINE 4 MONOOXYGENASE; TETRAHYDROBIOPTERIN;

EID: 79960842692     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2011.04.014     Document Type: Article
Times cited : (3)

References (5)
  • 3
    • 0032980507 scopus 로고    scopus 로고
    • A model of human phenylalanine metabolism in normal subjects and in phenylketonuric patients
    • Kaufman S. A model of human phenylalanine metabolism in normal subjects and in phenylketonuric patients. Proc. Natl. Acad. Sci. 1999, 96:3160-3164.
    • (1999) Proc. Natl. Acad. Sci. , vol.96 , pp. 3160-3164
    • Kaufman, S.1
  • 4
    • 0034820109 scopus 로고    scopus 로고
    • Tyrosine supplementation in phenylketonuria: diurnal blood tyrosine levels and presumptive brain influx of tyrosine and other large neutral amino acids
    • Kalsner L.R., Rohr F.J., Strauss K.A., Korson M.S., Levy H.L. Tyrosine supplementation in phenylketonuria: diurnal blood tyrosine levels and presumptive brain influx of tyrosine and other large neutral amino acids. J. Pediatr. 2001, 139:421-427.
    • (2001) J. Pediatr. , vol.139 , pp. 421-427
    • Kalsner, L.R.1    Rohr, F.J.2    Strauss, K.A.3    Korson, M.S.4    Levy, H.L.5
  • 5
    • 34547697475 scopus 로고    scopus 로고
    • Efficacy of sapropterin dihydrochloride (tetrahydrobiopterin,6R-BH4) for reduction of phenylalanine concentration in patients with phenylketonuria: a phase III randomised placebo-controlled study
    • Sapropterin Research Group
    • Levy H.L., Milanowski A., Chakrapani A., Cleary M., Lee P., Trefz F.K., Whitley C.B., Feillet F., Feigenbaum A.S., Bebchuk J.D., Christ-Schmidt H., Dorenbaum A. Efficacy of sapropterin dihydrochloride (tetrahydrobiopterin,6R-BH4) for reduction of phenylalanine concentration in patients with phenylketonuria: a phase III randomised placebo-controlled study. Lancet 2007, 370:504-510. Sapropterin Research Group.
    • (2007) Lancet , vol.370 , pp. 504-510
    • Levy, H.L.1    Milanowski, A.2    Chakrapani, A.3    Cleary, M.4    Lee, P.5    Trefz, F.K.6    Whitley, C.B.7    Feillet, F.8    Feigenbaum, A.S.9    Bebchuk, J.D.10    Christ-Schmidt, H.11    Dorenbaum, A.12


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.