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Pkhd1(cyli/cyli) mice have altered renal Pkhd1 mRNA processing and hormonally sensitive liver disease

ABSTRACT: Autosomal-recessive polycystic kidney disease (ARPKD; MIM #263200) is a severe, hereditary, hepato-renal fibrocystic disorder that causes early childhood morbidity and mortality. Mutations in the polycystic kidney and hepatic disease 1 (PKHD1) gene, which encodes the protein fibrocystin/po...

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Detalles Bibliográficos
Autores principales: Yang, Chaozhe, Harafuji, Naoe, Caldovic, Ljubica, Yu, Weiying, Boddu, Ravindra, Bhattacharya, Surajit, Barseghyan, Hayk, Gordish-Dressman, Heather, Foreman, Oded, Bebok, Zsuzsa, Eicher, Eva M., Guay-Woodford, Lisa M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Berlin Heidelberg 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10482757/
https://www.ncbi.nlm.nih.gov/pubmed/37584738
http://dx.doi.org/10.1007/s00109-023-02351-2