A North American team has characterised the liver phenotype of a zebrafish model of myotubular myopathy.
- The loss-of-function anomaly in mtm1 results in impaired bile flow and structural anomalies in the bile canaliculi, with inadequate endosomal trafficking of bile transporters.
- Hepatocyte re-expression of myotubularin 1 is sufficient to at least partially restore these abnormalities.
- Two dynamin 2 inhibitors, dynasore and dyngo-4a, partially restore bile flow and the position of bile transporters at the membrane of the bile canaliculi.