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

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

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http://www.wikidata.org/entity/Q43152626
Activation of phenylalanine hydroxylase induces positive cooperativity toward the natural cofactorA new model for allosteric regulation of phenylalanine hydroxylase: implications for disease and therapeuticsPharmacological chaperoning: a primer on mechanism and pharmacologyProtein homeostasis disorders of key enzymes of amino acids metabolism: mutation-induced protein kinetic destabilization and new therapeutic strategies.Influence of PAH Genotype on Sapropterin Response in PKU: Results of a Single-Center Cohort Study.Novel pharmacological chaperones that correct phenylketonuria in mice.Up to date knowledge on different treatment strategies for phenylketonuria.Tetrahydrobiopterin responsiveness in phenylketonuria: prediction with the 48-hour loading test and genotype.Innovative strategies to treat protein misfolding in inborn errors of metabolism: pharmacological chaperones and proteostasis regulators.Recombinant heteromeric phenylalanine monooxygenase and the oxygenation of carbon and sulfur substrates.Mapping the functional landscape of frequent phenylalanine hydroxylase (PAH) genotypes promotes personalised medicine in phenylketonuria.Long-term pharmacological management of phenylketonuria, including patients below the age of 4 years.Sapropterin treatment does not enhance the health-related quality of life of patients with phenylketonuria and their parents.Pharmacological Chaperones: Beyond Conformational Disorders.Prevalence of tetrahydrobiopterine (BH4)-responsive alleles among Austrian patients with PAH deficiency: comprehensive results from molecular analysis in 147 patients.
P2860

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

Item
http://www.wikidata.org/entity/Q43152626
description
2010 nî lūn-bûn
@nan
2010年の論文
@ja
2010年学术文章
@wuu
2010年学术文章
@zh
2010年学术文章
@zh-cn
2010年学术文章
@zh-hans
2010年学术文章
@zh-my
2010年学术文章
@zh-sg
2010年學術文章
@yue
2010年學術文章
@zh-hant
name
Pahenu1 is a mouse model for t ...... l chaperone mechanism in vivo.
@en
Pahenu1 is a mouse model for t ...... l chaperone mechanism in vivo.
@nl
type
label
Pahenu1 is a mouse model for t ...... l chaperone mechanism in vivo.
@en
Pahenu1 is a mouse model for t ...... l chaperone mechanism in vivo.
@nl
prefLabel
Pahenu1 is a mouse model for t ...... l chaperone mechanism in vivo.
@en
Pahenu1 is a mouse model for t ...... l chaperone mechanism in vivo.
@nl
P1433
P1476
P2093
P304
P31
P356
P433
P478
P577
P5875
P698
P921
P356
P1433
P1476
Pahenu1 is a mouse model for t ...... l chaperone mechanism in vivo.
@en
P2093
P304
P31
P356
P433
P478
P577
P5875
P698
P921