Fibrocystin (IPR028839)

Short name: PKHD1

Overlapping homologous superfamilies


Family relationships



Fibrocystin, also known as polyductin or polycystic kidney and hepatic disease 1 protein (PKHD1), may be required for correct bipolar cell division through the regulation of centrosome duplication and mitotic spindle assembly [PMID: 20554582]. There are several alternative forms of fibrocystin which are expressed in plasma membrane, primary cilium, and cytoplasm [PMID: 15458427]. The mature cleaved form of fibrocystin is found in exosome-like vesicles (ELVs) in mouse urine. These fibrocystin-containing ELVs can rapidly associate with primary cilia in the kidney [PMID: 22080423].

Mutations in fibrocystin gene cause polycystic kidney disease, autosomal recessive (ARPKD), which is a severe form of polycystic kidney disease affecting the kidneys and the hepatic biliary tract [PMID: 11919560].

GO terms

Biological Process

GO:0010824 regulation of centrosome duplication

Molecular Function

No terms assigned in this category.

Cellular Component

No terms assigned in this category.

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.