Pub. Date : 2012 Sep
PMID : 22609309
4 Functional Relationships(s)Download |
Sentence | Compound Name | Protein Name | Organism |
| 1 | After an in vitro study in a hepatocellular polarized line, we tested 4-PB treatment in a child with a homozygous p.T1210P BSEP mutation. | 4-phenylbutyric acid | ATP binding cassette subfamily B member 11 | Homo sapiens |
| 2 | 4-PB as well as incubation at 27 C partially corrected Bsep(T1210P)-GFP targeting to the canalicular membrane, while combined treatments resulted in normal canalicular localization. | 4-phenylbutyric acid | ATP binding cassette subfamily B member 11 | Homo sapiens |
| 3 | CONCLUSIONS: The results illustrate for the first time the therapeutic potential of a clinically approved chaperone drug in a selected patient with PFIC2 and support that bile secretion improvement might be due to the ability of 4-PB to retarget mutated BSEP. | 4-phenylbutyric acid | ATP binding cassette subfamily B member 11 | Homo sapiens |
| 4 | CONCLUSIONS: The results illustrate for the first time the therapeutic potential of a clinically approved chaperone drug in a selected patient with PFIC2 and support that bile secretion improvement might be due to the ability of 4-PB to retarget mutated BSEP. | 4-phenylbutyric acid | ATP binding cassette subfamily B member 11 | Homo sapiens |