DATASHEET
Host:
Rabbit
Target Protein:
CCDC39
Immunogen Range:
651-750/941
Clonality:
Polyclonal
Isotype:
IgG
Entrez Gene:
339829
Source:
KLH conjugated synthetic peptide derived from human CCDC39
Purification:
Purified by Protein A.
Storage Buffer:
0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Storage:
Shipped at 4C. Store at -20C for one year. 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.Tissue specificity:Mainly expressed in nasal brushings and, to a lesser extent, in lungs and testis.Involvement in disease:Defects in CCDC39 are the cause of primary ciliary dyskinesia type 14 (CILD14) . 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.
VALIDATION IMAGES
Lane 1: DU145 cell lysates probed with CCDC39 Polyclonal Antibody, Unconjugated (bs-8096R) at 1:1000 dilution and 4˚C overnight incubation. Followed by conjugated secondary antibody incubation at 1:20000 for 60 min at 37˚C.
Lane 1: HL-60 cell lysates probed with CCDC39 Polyclonal Antibody, Unconjugated (bs-8096R) at 1:1000 dilution and 4˚C overnight incubation. Followed by conjugated secondary antibody incubation at 1:20000 for 60 min at 37˚C.