The C-type lectins are a superfamily of proteins that recognize a broad repertoire of ligands
and that regulate a diverse range of physiological functions. Most research attention has …

Primary cilia are specialized sensory organelles that protrude from the apical surface of most
cell types. Over the past two decades, they have been found to play important roles in tissue …

Data indicate significant phenotypic and genotypic overlap, plus a common pathogenesis,
between two groups of inherited disorders, autosomal dominant polycystic kidney diseases …

Dominantly inherited isolated polycystic liver disease (PCLD) consists of liver cysts that are
radiologically and pathologically identical to those seen in autosomal dominant polycystic …

Clinical vignette A 41-year old female underwent a computed tomography (CT) scan in 2010
because of symptoms suggestive of appendicitis. Incidentally, multiple liver lesions …

In the last 25 years, a number of animal models, mainly rodents, have been generated with
the goal to mimic cholestatic liver injuries and, thus, to provide in vivo tools to investigate the …

The heterotrimeric Sec61 complex and the dimeric Sec62/Sec63 complex are located in the
membrane of the human endoplasmic reticulum (ER) and play a central role in translocation …

Autosomal dominant polycystic kidney disease is the most common monogenic cause of
ESKD. Genetic studies from patients and animal models have informed disease …

Autosomal dominant polycystic kidney disease (ADPKD) is the leading genetic cause of end
stage renal disease characterized by progressive expansion of kidney cysts. To better …

Autosomal-dominant polycystic kidney disease (ADPKD) is a disease of defective tissue
homeostasis resulting in active remodeling of nephrons and bile ducts to form fluid-filled …