Nephrocystin-4 (IPR029775)

Short name: NPHP4

Overlapping homologous superfamilies


Family relationships



All nephrocystin proteins (NPHPs) localise to primary cilia, which are critical regulators of several cell signalling pathways for embryonic development and tissue homeostasis.

Nephrocystin-4 (NPHP4) localises to the basal body [PMID: 15661758] and is thought to be involved in basal body docking and organising ciliary trafficking [PMID: 21422230]. It is a negative regulator of canonical Wnt signalling [PMID: 21498478, PMID: 22654112]. Mutations in NPHP4 cause Nephronophthisis 4, an autosomal-recessive cystic kidney disease that belongs to a broad spectrum of disorders collectively termed ciliopathies [PMID: 12244321].

GO terms

Biological Process

GO:0090090 negative regulation of canonical Wnt signaling pathway

Molecular Function

No terms assigned in this category.

Cellular Component

GO:0005856 cytoskeleton
GO:0097730 non-motile cilium

Contributing signatures

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