Target intelligence / Profile preview

WD repeat-containing protein 35 (WDR35)

Target
WDR35
Molecular classification
WD repeat protein family, Intraflagellar transport protein, Component of IFT-A complex, Other
01

Overview

WD repeat-containing protein 35 (WDR35) is a core component of the intraflagellar transport A (IFT-A) complex, which is essential for the assembly and maintenance of primary cilia by mediating retrograde transport of proteins and membrane cargo from the ciliary tip to the base[1][3][4][6]. As a member of the WD repeat protein family, it facilitates multiprotein complex formation and has roles in cell cycle progression, apoptosis, and gene regulation[1][5][7]. WDR35 is crucial for proper Sonic Hedgehog signaling and developmental processes, especially during embryogenesis. Mutations in WDR35 disrupt ciliary function, manifesting as a spectrum of skeletal and multisystem ciliopathies, most notably cranioectodermal dysplasia (Sensenbrenner syndrome) and short-rib thoracic dysplasia[3][5]. No approved drugs specifically target WDR35; its dysfunction emerges primarily as a genetic disease mechanism rather than a current therapeutic target[1][5].

Other names
IFT121WD repeat domain 35KIAA1336MGC33196IFTA1FAP118CFAP118Intraflagellar transport protein 121 homologCED2SRTD7naofen
02

Biological functions

Ciliogenesis and ciliary protein traffickingRetrograde ciliary transport (intraflagellar transport)Cell cycle progressionSignal transductionApoptosisGene regulation
03

Disease associations

Skeletal ciliopathies (e.g., Sensenbrenner syndrome/cranioectodermal dysplasia)Short-rib thoracic dysplasia with or without polydactylyEllis–van Creveld syndromeCoronary artery disease (as a potential modifier)Other
04

Safety considerations

Mutations in WDR35 result in defective ciliogenesis, leading to severe multisystem syndromes affecting skeletal, craniofacial, ectodermal, and visceral systems[1][3][5].

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