CCDC39 Rabbit pAb
- 产品详情
- 实验流程
Application
| WB |
|---|---|
| Primary Accession | Q9UFE4 |
| Other Accession | Q9UFE4 |
| Reactivity | Human |
| Predicted | Rat, Mouse, Rabbit, Horse |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 109901 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human CCDC39 |
| Epitope Specificity | 651-750/941 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SIMILARITY | Belongs to the CCDC39 family. |
| DISEASE | Defects in CCDC39 are the cause of primary ciliary dyskinesia type 14 (CILD14) [MIM:613807]. 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. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | 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. |
| Gene ID | 339829 |
|---|---|
| Other Names | CFAP59; CILD14; FAP59; 4921507O14Rik; D3Ertd789e; b2b1304Clo; b2b1735Clo; b2b2025.1Clo; prh; CCD39_CANLF; CCDC39; CCD39_HUMAN; CCD39_MOUSE; coiled-coil domain 39 molecular ruler complex subunit; coiled-coil domain containing 39 |
| Dilution | WB=1:500-2000 |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | CCDC39 (HGNC:25244) |
|---|---|
| Function | Required for assembly of dynein regulatory complex (DRC) and inner dynein arm (IDA) complexes, which are responsible for ciliary beat regulation, thereby playing a central role in motility in cilia and flagella (PubMed:21131972). Probably acts together with CCDC40 to form a molecular ruler that determines the 96 nanometer (nm) repeat length and arrangements of components in cilia and flagella (By similarity). Not required for outer dynein arm complexes assembly (PubMed:21131972). |
| Cellular Location | Cytoplasm, cytoskeleton, cilium axoneme. Note=CCDC40 is required for localization to axonemes. |
| Tissue Location | Mainly expressed in nasal brushings and, to a lesser extent, in lungs and testis. |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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