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The Predictive Value of Genetic Analyses in the Diagnosis of Tetrahydrobiopterin (BH4)-Responsiveness in Chinese Phenylalanine Hydroxylase Deficiency Patients

Molecular characterization of PAH deficiency has been proven essential in establishing treatment options. We examine the diagnostic accuracy of two genetic assays to predict BH4 responsiveness: to determine whether the AV sum test or mutation-status assessment test can obviate the need for BH4 loadi...

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Dettagli Bibliografici
Pubblicato in:Sci Rep
Autori principali: Zhu, Tianwen, Ye, Jun, Han, Lianshu, Qiu, Wenjuan, Zhang, Huiwen, Liang, Lili, Gu, Xuefan
Natura: Artigo
Lingua:Inglês
Pubblicazione: Nature Publishing Group UK 2017
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC5533732/
https://ncbi.nlm.nih.gov/pubmed/28754886
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/s41598-017-06462-y
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