Cystic kidneys are common causes of end-stage renal disease, both in children and in
adults. Autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive …

The intramembrane protease γ-secretase is a hetero-tetrameric protein complex with
presenilin as the catalytic subunit and cleaves its membrane protein substrates within their …

The Alzheimer's disease (AD)-associated amyloid-β protein precursor (AβPP) is cleaved by
α-, β-, and presenilin (PS)/γ-secretases through sequential regulated proteolysis. These …

A number of inherited disorders result in renal cyst development. The most common form,
autosomal dominant polycystic kidney disease (ADPKD), is a disorder most often diagnosed …

Proteins associated with autosomal dominant and autosomal recessive polycystic kidney
disease (polycystin-1, polycystin-2, and fibrocystin) localize to various subcellular …

Polycystic kidney disease (PKD) is an inherited disorder that is characterized by the
accumulation of cysts in the renal parenchyma and progressive decline in renal function …

Autosomal recessive polycystic kidney disease (ARPKD) is a hepatorenal fibrocystic
disorder that is characterized by enlarged kidneys with progressive loss of renal function …

γ-Secretase is a promiscuous protease that cleaves bitopic membrane proteins within the
lipid bilayer. Elucidating both the mechanistic basis of γ-secretase proteolysis and the …

Autosomal dominant polycystic liver disease results from mutations in PRKCSH or SEC63.
The respective gene products, glucosidase IIβ and SEC63p, function in protein translocation …

Bile duct epithelial cells, also known as cholangiocytes, regulate the composition of bile and
its flow. Acquired, congenital and genetic dysfunctions in these cells give rise to a set of …