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:
Aqueous buffered solution containing 0.01M TBS (pH 7.4) 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.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.
Conjugation:
ALEXA FLUOR® 488
Excitation/ Emission:
490nm/525nm
Size:
100ul
Concentration:
1ug/ul
Applications:
WB(1:300-5000)
110
Human
Predicted Cross Reactive Species:
Mouse
Rat
Cow
Horse
Rabbit
For research use only. Not intended for diagnostic or therapeutic use.