WDR35 Antibody, FITC conjugated
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Description
Biological Context of WDR35
WDR35, also known as IFT121, is a core component of the intraflagellar transport-A (IFT-A) complex. It facilitates retrograde cargo transport in cilia and is essential for:
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Hedgehog (Hh) Signaling: Mediates ciliary localization of Smoothened (SMO) and EvC complex proteins .
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Protein Trafficking: Acts as a COPI-like coat for vesicular delivery of ciliary membrane proteins .
Mutations in WDR35 are linked to ciliopathies such as cranioectodermal dysplasia (CED) and Ellis-van Creveld syndrome .
Immunofluorescence (IF) and ELISA
The FITC conjugate enables precise localization of WDR35 in cilia and cytoplasmic compartments. Studies using this antibody have revealed:
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Accumulation of IFT-B complex proteins in Wdr35 mutant cilia .
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Disrupted ciliary entry of SMO and EvC proteins in Wdr35 knockout models .
Functional Insights
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Ciliary Coat Function: WDR35 binds lipids and facilitates vesicle fusion to cilia, akin to COPI coatomer subunits .
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Protein Stability: Pathogenic WDR35 variants destabilize IFT-A components (e.g., IFT43) and impair ciliary trafficking .
Comparative Data from Mutant Models
Handling and Technical Notes
Product Specs
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Lead Time
Generally, we can ship the products within 1-3 business days after receiving your order. Delivery time may vary depending on the purchasing method or location. For specific delivery time, please contact your local distributor.
Synonyms
Intraflagellar transport protein 121 homolog antibody; KIAA1336 antibody; MGC33196 antibody; Naofen antibody; WD repeat domain 35 antibody; WD repeat-containing protein 35 antibody; WDR35 antibody; WDR35_HUMAN antibody
Target Names
WDR35
Uniprot No.
Target Background
Function
WDR35, as a component of the IFT complex A (IFT-A), plays a crucial role in retrograde ciliary transport and the entry of G protein-coupled receptors (GPCRs) into cilia. This involvement contributes to ciliogenesis and the trafficking of ciliary proteins. Moreover, WDR35 might promote CASP3 activation and TNF-stimulated apoptosis.
Gene References Into Functions
- Homozygous missense mutations in the WDR35 gene are associated with multiple congenital anomalies, including brain malformations and skeletal dysplasia suggestive of cranioectodermal dysplasia ciliopathy. PMID: 29174089
- The analysis of the Sensenbrenner syndrome patient in this study provides additional clinical data and expands the molecular spectrum of Sensenbrenner syndrome. Furthermore, the two variants identified in the proband offer further evidence that WDR35 mutations are the most common cause of this rare syndrome. PMID: 29134781
- A differential diagnosis of Sensenbrenner Syndrome was made after a novel homozygous missense mutation in WDR35 was identified in a patient with an initial diagnosis of Jeune syndrome. PMID: 28870638
- Wdr35 regulates cilium assembly by selectively regulating the transport of distinct cargoes. PMID: 27806291
- Psychomotor development was apparently normal. Molecular analysis in one of the affected individuals identified compound heterozygosity for a nonsense (c.1922T>G, p.(Leu641*)) and missense (c.2522A>T, p.(Asp841Val)) variants in WDR35. We PMID: 28332779
- Splicing variants in WDR35, and possibly in other IFT-A components, underlie a number of Ellis-van Creveld syndrome cases by disrupting targeting of both the EvC complex and Smoothened to cilia. PMID: 25908617
- report on the detection of novel WDR35 mutations in two unrelated cranioectodermal dysplasia patients PMID: 22486404
- A pathogenic WDR35 mutation was identified in a family with a complex clinical presentation that includes significant overlap of the phenotypes described in Sensenbrenner syndrome and the unclassified short-rib polydactyly syndromes. PMID: 22987818
- Through structural modeling, we demonstrate that WDR35 has strong homology to the COPI coatamers involved in vesicular trafficking and that short-rib polydactyly mutations affect key structural elements in WDR35. PMID: 21473986
- WDR35 is homologous to TULP4 (from the Tubby superfamily) and has previously been characterized as an intraflagellar transport component, confirming that Sensenbrenner syndrome is a ciliary disorder. PMID: 20817137
- These results indicate that naofen may function as a novel modulator activating caspase-3, and promoting TNF-alpha-stimulated apoptosis. PMID: 20193664
Database Links
Involvement In Disease
Cranioectodermal dysplasia 2 (CED2); Short-rib thoracic dysplasia 7 with or without polydactyly (SRTD7)
Subcellular Location
Cytoplasm, cytoskeleton, microtubule organizing center, centrosome. Cytoplasm, cytoskeleton, cilium axoneme. Cytoplasm, cytoskeleton, cilium basal body.
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