Literature DB >> 2334400

Mouse phenylalanine hydroxylase. Homology and divergence from human phenylalanine hydroxylase.

F D Ledley1, H E Grenett, B S Dunbar, S L Woo.   

Abstract

The laboratory mouse represents an important model for the study of phenylalanine metabolism and the pathochemistry of phenylketonuria, yet mouse phenylalanine hydroxylase (PAH) has not been extensively studied. We report the cloning and sequencing of a mouse PAH cDNA, the expression of enzymic activity from the mouse PAH cDNA clone and the identification of mouse PAH and human PAH by two-dimensional PAGE of liver samples. These data confirm the expected homology of mouse PAH and human PAH and suggest differences in the primary sequence and the phosphorylation state of the two enzymes.

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Year:  1990        PMID： 2334400      PMCID： PMC1131302          DOI： 10.1042/bj2670399

Source DB:  PubMed          Journal:  Biochem J        ISSN： 0264-6021            Impact factor:   3.857


  41 in total

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Journal:  Proc Natl Acad Sci U S A       Date:  1983-03       Impact factor: 11.205

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Journal:  Cell       Date:  1981-12       Impact factor: 41.582

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Journal:  J Mol Biol       Date:  1982-05-05       Impact factor: 5.469

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Authors:  N L Anderson; N G Anderson
Journal:  Anal Biochem       Date:  1978-04       Impact factor: 3.365

Review 5.  Phenylketonuria: epitome of human biochemical genetics (first of two parts).

Authors:  C R Scriver; C L Clow
Journal:  N Engl J Med       Date:  1980-12-04       Impact factor: 91.245

6.  Phenylketonuria: epitome of human biochemical genetics (second of two parts).

Authors:  C R Scriver; C L Clow
Journal:  N Engl J Med       Date:  1980-12-11       Impact factor: 91.245

7.  Amino acid sequence at the phosphorylated site of rat liver phenylalanine hydroxylase and phosphorylation of a corresponding synthetic peptide.

Authors:  M Wretborn; E Humble; U Ragnarsson; L Engström
Journal:  Biochem Biophys Res Commun       Date:  1980-03-28       Impact factor: 3.575

8.  Genetics of the mammalian phenylalanine hydroxylase system. Studies of human liver phenylalanine hydroxylase subunit structure and of mutations in phenylketonuria.

Authors:  K H Choo; R G Cotton; D M Danks; I G Jennings
Journal:  Biochem J       Date:  1979-08-01       Impact factor: 3.857

9.  Polysome immunoprecipitation of phenylalanine hydroxylase mRNA from rat liver and cloning of its cDNA.

Authors:  K J Robson; T Chandra; R T MacGillivray; S L Woo
Journal:  Proc Natl Acad Sci U S A       Date:  1982-08       Impact factor: 11.205

10.  Isolation of biologically active ribonucleic acid from sources enriched in ribonuclease.

Authors:  J M Chirgwin; A E Przybyla; R J MacDonald; W J Rutter
Journal:  Biochemistry       Date:  1979-11-27       Impact factor: 3.162
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  13 in total

Review 1.  Mouse chromosome 10.

Authors:  B A Taylor; M J Justice; R Reeves
Journal:  Mamm Genome       Date:  1991       Impact factor: 2.957

2.  Recombinant human phenylalanine hydroxylase is a substrate for the ubiquitin-conjugating enzyme system.

Authors:  A P Døskeland; T Flatmark
Journal:  Biochem J       Date:  1996-11-01       Impact factor: 3.857

Review 3.  Structure and function of the aromatic amino acid hydroxylases.

Authors:  S E Hufton; I G Jennings; R G Cotton
Journal:  Biochem J       Date:  1995-10-15       Impact factor: 3.857

4.  Complete and persistent phenotypic correction of phenylketonuria in mice by site-specific genome integration of murine phenylalanine hydroxylase cDNA.

Authors:  Li Chen; Savio L C Woo
Journal:  Proc Natl Acad Sci U S A       Date:  2005-10-17       Impact factor: 11.205

5.  Metabolic engineering as therapy for inborn errors of metabolism--development of mice with phenylalanine hydroxylase expression in muscle.

Authors:  C O Harding; K Wild; D Chang; A Messing; J A Wolff
Journal:  Gene Ther       Date:  1998-05       Impact factor: 5.250

6.  3-Hydroxy-3-methylglutaryl coenzyme A lyase (HL): cloning and characterization of a mouse liver HL cDNA and subchromosomal mapping of the human and mouse HL genes.

Authors:  S Wang; J H Nadeau; A Duncan; M F Robert; G Fontaine; K Schappert; K R Johnson; E Zietkiewicz; P Hruz; H Miziorko
Journal:  Mamm Genome       Date:  1993       Impact factor: 2.957

7.  A molecular genetic linkage map of mouse chromosome 10, including the Myb, S100b, Pah, Sl, and Ifg genes.

Authors:  A Shimizu; Y Sakai; K Ohno; S Masaki; R Kuwano; Y Takahashi; N Miyashita; T Watanabe
Journal:  Biochem Genet       Date:  1992-10       Impact factor: 1.890

8.  Phosphorylation of recombinant human phenylalanine hydroxylase: effect on catalytic activity, substrate activation and protection against non-specific cleavage of the fusion protein by restriction protease.

Authors:  A P Døskeland; A Martinez; P M Knappskog; T Flatmark
Journal:  Biochem J       Date:  1996-01-15       Impact factor: 3.857

9.  Expression of phenylalanine hydroxylase (PAH) in erythrogenic bone marrow does not correct hyperphenylalaninemia in Pah(enu2) mice.

Authors:  Cary O Harding; Mark Neff; Kelly Jones; Krzysztof Wild; Jon A Wolff
Journal:  J Gene Med       Date:  2003-11       Impact factor: 4.565

10.  Transgene-induced mutation of the murine steel locus.

Authors:  S A Keller; S Liptay; A Hajra; M H Meisler
Journal:  Proc Natl Acad Sci U S A       Date:  1990-12       Impact factor: 11.205
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