First published online November 10, 2005
Development 132, 2305e (2005)
© The Company of Biologists Limited
Intracellular traffic disruption: red light to bile flow
Reduced bile flow - cholestasis - is usually the result of liver injury but
is also seen in some inherited syndromes. In arthrogryposis-renal
dysfunction-cholestasis syndrome (ARC), which is caused by mutations in the
vacuolar sorting protein VPS33B, improper bile duct development in
the liver contributes to the cholestasis part of the syndrome. Now, Matthews
and co-workers describe a zebrafish model of ARC. They report that
vps33b-deficient zebrafish larvae have poorly developed bile ducts
and impaired lipid absorption, as do people with ARC, but that they lack the
characteristic motor axon or renal defects (see p.
5295). In addition,
they identify vps33b as a downstream target gene of the transcription
factor hnf6, which regulates bile duct development in zebrafish.
Further elucidation of the role of VPS33B in biliary development will require
the identification of the proteins whose intracellular trafficking is
disrupted by its loss.
Related articles in Development:
- Zebrafish vps33b, an ortholog of the gene responsible for human arthrogryposis-renal dysfunction-cholestasis syndrome, regulates biliary development downstream of the onecut transcription factor hnf6
- Randolph P. Matthews, Nicolas Plumb-Rudewiez, Kristin Lorent, Paul Gissen, Colin A. Johnson, Frederic Lemaigre, and Michael Pack
Development 2005 132: 5295-5306.
[Abstract]
[Full Text]
