Pharmaceutical compositions comprising an inorganic pyrophosphate (PPi) in a form that renders the PPi accessible to ABC proteins when administered to a subject in vivo, as well as use of the compositions for treating subjects having a disease or disorder associated with inappropriate or inadequate ABC protein activity (e.g., cystic fibrosis, multi drug resistance, Zellweger's Syndrome) is disclosed.

Las composiciones farmacéuticas que abarcan un pirofosfato inorgánico (PPi) en una forma que haga el PPi accesible a las proteínas del ABC cuando está administrada a un in vivo sujeto, tan bien como el uso de las composiciones para tratar los temas que tienen una enfermedad o un desorden asociado a la actividad inadecuada o inadecuada de la proteína del ABC (e.g., fibrosis enquistada, multi resistencia de la droga, síndrome de Zellweger) se divulgan.

 
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