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2. PKU mutation (D143G) associated with an apparent high residual enzyme activity: expression of a kinetic variant form of phenylalanine hydroxylase in three different systems. Knappskog PM; Eiken HG; Martínez A; Bruland O; Apold J; Flatmark T Hum Mutat; 1996; 8(3):236-46. PubMed ID: 8889583 [TBL] [Abstract][Full Text] [Related]
3. Degradation rates differ between mutant and wild-type forms of phenylalanine hydroxylase expressed in vitro. Waters PJ; Scriver CR; Parniak MA Biochem Soc Trans; 1998 Aug; 26(3):S276. PubMed ID: 9765995 [No Abstract] [Full Text] [Related]
4. Phenylketonuria in China: identification and characterization of three novel nucleotide substitutions in the human phenylalanine hydroxylase gene. Li J; Eisensmith RC; Wang T; Lo WH; Huang SZ; Zeng YT; Yuan LF; Liu SR; Woo SL Hum Mutat; 1994; 3(3):312-4. PubMed ID: 8019568 [No Abstract] [Full Text] [Related]
5. Phenylalanine and tyrosine metabolism in phenylketonuria heterozygotes: influence of different phenylalanine hydroxylase mutations. Spada M; Dianzani I; Bonetti G; Biondi A; Leone L; Giannattasio S; Ponzone A J Inherit Metab Dis; 1998 Jun; 21(3):236-9. PubMed ID: 9686365 [No Abstract] [Full Text] [Related]
6. The V388M mutation results in a kinetic variant form of phenylalanine hydroxylase. Leandro P; Rivera I; Lechner MC; de Almeida IT; Konecki D Mol Genet Metab; 2000 Mar; 69(3):204-12. PubMed ID: 10767175 [TBL] [Abstract][Full Text] [Related]
7. Five novel missense mutations of the phenylalanine hydroxylase gene in phenylketonuria. Bénit P; Rey F; Melle D; Munnich A; Rey J Hum Mutat; 1994; 4(3):229-31. PubMed ID: 7833954 [No Abstract] [Full Text] [Related]
8. The mutations and VNTRs in the phenylalanine hydroxylase gene of phenylketonuria in St Petersburg. Baranovskaya S; Shevtsov S; Maksimova S; Kuzmin A; Schwartz E J Inherit Metab Dis; 1996; 19(5):705. PubMed ID: 8892033 [No Abstract] [Full Text] [Related]
9. PKU mutation G46S is associated with increased aggregation and degradation of the phenylalanine hydroxylase enzyme. Eiken HG; Knappskog PM; Apold J; Flatmark T Hum Mutat; 1996; 7(3):228-38. PubMed ID: 8829656 [TBL] [Abstract][Full Text] [Related]
10. Observations indicating the nature of the mutation in phenylketonuria. Choo KH; Cotton RG; Jennings IG; Danks DM J Inherit Metab Dis; 1980; 2(4):79-84. PubMed ID: 6796764 [TBL] [Abstract][Full Text] [Related]
11. Connecting mutant phenylalanine hydroxylase with phenylketonuria. Yan S; Wu G J Clin Monit Comput; 2008 Oct; 22(5):333-42. PubMed ID: 18773304 [TBL] [Abstract][Full Text] [Related]
12. Genetics of the mammalian phenylalanine hydroxylase system. Studies of human liver phenylalanine hydroxylase subunit structure and of mutations in phenylketonuria. Choo KH; Cotton RG; Danks DM; Jennings IG Biochem J; 1979 Aug; 181(2):285-94. PubMed ID: 496890 [TBL] [Abstract][Full Text] [Related]
13. Phenylalanine hydroxylase gene: a novel splice mutation in intron 2 in two German and Polish families with severe phenylketonuria. Zygulska M; Eigel A; Pietrzyk JJ; Horst J Hum Mutat; 1993; 2(3):238-9. PubMed ID: 8364593 [No Abstract] [Full Text] [Related]
14. [Genetical heterogeneity of phenylketonuria]. Annenkov GA Vopr Med Khim; 1982; 28(3):62-70. PubMed ID: 7048735 [TBL] [Abstract][Full Text] [Related]
15. Phenylketonuria mutant alleles in different populations: missense mutation in exon 7 of phenylalanine hydroxylase gene. Takarada Y; Kalanin J; Yamashita K; Ohtsuka N; Kagawa S; Matsuoka A Clin Chem; 1993 Nov; 39(11 Pt 1):2354-5. PubMed ID: 8222245 [No Abstract] [Full Text] [Related]
16. CpG hotspot causes second mutation in codon 408 of the phenylalanine hydroxylase gene. Ramus SJ; Forrest SM; Saleeba JA; Cotton RG Hum Genet; 1992; 90(1-2):147-8. PubMed ID: 1358783 [TBL] [Abstract][Full Text] [Related]
17. Tetrahydrobiopterin for patients with phenylketonuria. Pey AL; Martinez A Lancet; 2007 Aug; 370(9586):462-3. PubMed ID: 17693159 [No Abstract] [Full Text] [Related]
18. In vitro expression analysis of R68G and R68S mutations in phenylalanine hydroxylase gene. Zekanowsk C; Perez B; Desviat LR; Wiszniewski W; Ugarte M Acta Biochim Pol; 2000; 47(2):365-9. PubMed ID: 11051201 [TBL] [Abstract][Full Text] [Related]
19. Phenylalanine hydroxylase expression in liver of a fetus with phenylketonuria. Ledley FD; Koch R; Jew K; Beaudet A; O'Brien WE; Bartos DP; Woo SL J Pediatr; 1988 Sep; 113(3):463-8. PubMed ID: 2900886 [TBL] [Abstract][Full Text] [Related]
20. Focus on the molecular genetics of phenylketonuria. Zschocke J Hum Mutat; 2003 Apr; 21(4):331-2. PubMed ID: 12655542 [No Abstract] [Full Text] [Related] [Next] [New Search]