bs-8091R-BF488

DATASHEET

Host: Rabbit

Target Protein: CCDC40

Immunogen Range: 851-950/1142

Clonality: Polyclonal

Isotype: IgG

Entrez Gene: 55036

Source: KLH conjugated synthetic peptide derived from human CCDC4

Purification: Purified by Protein A.

Storage Buffer: Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Storage: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Background:

Required for assembly of dynein regulatory complex (DRC) and inner dynein arm complexes, which are responsible for ciliary beat regulation, thereby playing a central role in motility in cilia and flagella. Not required for outer dynein arm complexes assembly. Required for axonemal recruitment of CCDC39.Involvement in disease:Defects in CCDC40 are the cause of primary ciliary dyskinesia type 15 (CILD15) . A disorder characterized by abnormalities of motile cilia. Respiratory infections leading to chronic inflammation and bronchiectasis are recurrent, due to defects in the respiratory cilia; reduced fertility is often observed in male patients due to abnormalities of sperm tails. Half of the patients exhibit randomization of left-right body asymmetry and situs inversus, due to dysfunction of monocilia at the embryonic node. Primary ciliary dyskinesia associated with situs inversus is referred to as Kartagener syndrome.

Conjugation: AbBy Fluor-488

Excitation/ Emission: 499nm/519nm

Size: 100µL

Concentration: 1ug/ul

Applications: IF(IHC-P)(1:50-200)
IF(IHC-F)(1:50-200)
IF(ICC)(1:50-200)

Predicted Molecular Weight: 130

Predicted Cross Reactive Species: Human
Mouse
Rat
Dog
Cow
Sheep
Pig
Horse

For research use only. Not intended for diagnostic or therapeutic use.