Pahenu1 is a mouse model for tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency and promotes analysis of the pharmacological chaperone mechanism in vivo.

Søren W Gersting; Florian Lagler; Anna Eichinger; Kristina F Kemter; Marta K Danecka; Dunja D Messing; Michael Staudigl; Katharina A Domdey; Clemens Zsifkovits; Ralph Fingerhut; Hartmut Glossmann; Adelbert A Roscher; Ania C Muntau

doi:10.1093/hmg/ddq085

Pahenu1 is a mouse model for tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency and promotes analysis of the pharmacological chaperone mechanism in vivo.

Søren W Gersting, Florian Lagler (Co-author), Anna Eichinger, Kristina F Kemter, Marta K Danecka, Dunja D Messing, Michael Staudigl, Katharina A Domdey, Clemens Zsifkovits, Ralph Fingerhut, Hartmut Glossmann, Adelbert A Roscher, Ania C Muntau

Research output: Contribution to journal › Original Article › peer-review

38 Citations (Web of Science)

Original language	English
Pages (from-to)	2039-2049
Journal	HUMAN MOLECULAR GENETICS
Volume	19
Issue number	10
DOIs	https://doi.org/10.1093/hmg/ddq085
Publication status	Published - 2010

Keywords

NEURODEGENERATIVE DISEASES
CONFORMATIONAL TRANSITION
MILD PHENYLKETONURIA
ESCHERICHIA-COLI
PROTEIN
MUTATIONS
HYPERPHENYLALANINEMIA
EXPRESSION
PHOSPHORYLATION
PROTEOSTASIS

Access to Document

10.1093/hmg/ddq085

Cite this

Gersting, S. W., Lagler, F., Eichinger, A., Kemter, K. F., Danecka, M. K., Messing, D. D., Staudigl, M., Domdey, K. A., Zsifkovits, C., Fingerhut, R., Glossmann, H., Roscher, A. A., & Muntau, A. C. (2010). Pahenu1 is a mouse model for tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency and promotes analysis of the pharmacological chaperone mechanism in vivo. HUMAN MOLECULAR GENETICS , 19(10), 2039-2049. https://doi.org/10.1093/hmg/ddq085

Gersting, Søren W ; Lagler, Florian ; Eichinger, Anna et al. / Pahenu1 is a mouse model for tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency and promotes analysis of the pharmacological chaperone mechanism in vivo. In: HUMAN MOLECULAR GENETICS 2010 ; Vol. 19, No. 10. pp. 2039-2049.

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title = "Pahenu1 is a mouse model for tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency and promotes analysis of the pharmacological chaperone mechanism in vivo.",

keywords = "NEURODEGENERATIVE DISEASES, CONFORMATIONAL TRANSITION, MILD PHENYLKETONURIA, ESCHERICHIA-COLI, PROTEIN, MUTATIONS, HYPERPHENYLALANINEMIA, EXPRESSION, PHOSPHORYLATION, PROTEOSTASIS",

author = "Gersting, {S{\o}ren W} and Florian Lagler and Anna Eichinger and Kemter, {Kristina F} and Danecka, {Marta K} and Messing, {Dunja D} and Michael Staudigl and Domdey, {Katharina A} and Clemens Zsifkovits and Ralph Fingerhut and Hartmut Glossmann and Roscher, {Adelbert A} and Muntau, {Ania C}",

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doi = "10.1093/hmg/ddq085",

language = "English",

volume = "19",

pages = "2039--2049",

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Gersting, SW, Lagler, F, Eichinger, A, Kemter, KF, Danecka, MK, Messing, DD, Staudigl, M, Domdey, KA, Zsifkovits, C, Fingerhut, R, Glossmann, H, Roscher, AA & Muntau, AC 2010, 'Pahenu1 is a mouse model for tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency and promotes analysis of the pharmacological chaperone mechanism in vivo.', HUMAN MOLECULAR GENETICS , vol. 19, no. 10, pp. 2039-2049. https://doi.org/10.1093/hmg/ddq085

Pahenu1 is a mouse model for tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency and promotes analysis of the pharmacological chaperone mechanism in vivo. / Gersting, Søren W; Lagler, Florian (Co-author); Eichinger, Anna et al.
In: HUMAN MOLECULAR GENETICS , Vol. 19, No. 10, 2010, p. 2039-2049.

Research output: Contribution to journal › Original Article › peer-review

TY - JOUR

T1 - Pahenu1 is a mouse model for tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency and promotes analysis of the pharmacological chaperone mechanism in vivo.

AU - Gersting, Søren W

AU - Lagler, Florian

AU - Eichinger, Anna

AU - Kemter, Kristina F

AU - Danecka, Marta K

AU - Messing, Dunja D

AU - Staudigl, Michael

AU - Domdey, Katharina A

AU - Zsifkovits, Clemens

AU - Fingerhut, Ralph

AU - Glossmann, Hartmut

AU - Roscher, Adelbert A

AU - Muntau, Ania C

PY - 2010

Y1 - 2010

KW - NEURODEGENERATIVE DISEASES

KW - CONFORMATIONAL TRANSITION

KW - MILD PHENYLKETONURIA

KW - ESCHERICHIA-COLI

KW - PROTEIN

KW - MUTATIONS

KW - HYPERPHENYLALANINEMIA

KW - EXPRESSION

KW - PHOSPHORYLATION

KW - PROTEOSTASIS

U2 - 10.1093/hmg/ddq085

DO - 10.1093/hmg/ddq085

M3 - Original Article

C2 - 20179079

SN - 0964-6906

VL - 19

SP - 2039

EP - 2049

JO - HUMAN MOLECULAR GENETICS

JF - HUMAN MOLECULAR GENETICS

IS - 10

ER -

Gersting SW, Lagler F, Eichinger A, Kemter KF, Danecka MK, Messing DD et al. Pahenu1 is a mouse model for tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency and promotes analysis of the pharmacological chaperone mechanism in vivo. HUMAN MOLECULAR GENETICS . 2010;19(10):2039-2049. doi: 10.1093/hmg/ddq085