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Monoallelic ABCC8 mutations are a common cause of diazoxide-unresponsive diffuse form of congenital hyperinsulinism

ABCC8 encodes a subunit of the β-cell potassium channel (K(ATP)) whose loss of function is responsible for congenital hyperinsulinism (CHI). Patients with two recessive mutations of ABCC8 typically have severe diffuse forms of CHI unresponsive to diazoxide. Some dominant ABCC8 mutations are responsi...

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Dettagli Bibliografici
Pubblicato in:Clin Genet
Autori principali: Saint-Martin, Cécile, Zhou, Qing, Martin, Gregory M., Vaury, Christelle, Leroy, Gwendoline, Arnoux, Jean-Baptiste, de Lonlay, Pascale, Shyng, Show-Ling, Bellanné-Chantelot, Christine
Natura: Artigo
Lingua:Inglês
Pubblicazione: 2014
Soggetti:
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC4229485/
https://ncbi.nlm.nih.gov/pubmed/24814349
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1111/cge.12428
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