| 產(chǎn)品編號 |
bs-8096R-BF647 |
| 英文名稱(chēng) |
Rabbit Anti-CCDC39/BF647 Conjugated antibody
|
| 中文名稱(chēng) |
BF647標記的卷曲螺旋結構域蛋白39抗體 |
| 別 名 |
CCD39_HUMAN; Ccdc39; Coiled-coil domain-containing protein 39. |
| 規格價(jià)格 |
100ul/2980元
購買(mǎi) 大包裝/詢(xún)價(jià) |
| 說(shuō) 明 書(shū) |
100ul
|
| 研究領(lǐng)域 |
細胞生物 免疫學(xué) 信號轉導 細胞骨架 |
| 抗體來(lái)源 |
Rabbit |
| 克隆類(lèi)型 |
Polyclonal |
| 交叉反應 |
(predicted: Human, Mouse, Rat, Cow, Horse, Rabbit, )
|
| 產(chǎn)品應用 |
IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 |
110kDa |
| 性 狀 |
Lyophilized or Liquid |
| 濃 度 |
1mg/ml |
| 免 疫 原 |
KLH conjugated synthetic peptide derived from human CCDC39 |
| 亞 型 |
IgG |
| 純化方法 |
affinity purified by Protein A |
| 儲 存 液 |
0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 |
Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. 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. |
| 產(chǎn)品介紹 |
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.
Function:
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.
Subcellular Location:
Cytoplasm, cytoskeleton, cilium axoneme. Note=CCDC40 is required for localization to axonemes.
Tissue Specificity:
Mainly expressed in nasal brushings and, to a lesser extent, in lungs and testis.
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.
Similarity:
Belongs to the CCDC39 family.
Database links:
Entrez Gene: 488089 Dog
Entrez Gene: 339829 Human
Entrez Gene: 51938 Mouse
Omim: 613798 Human
SwissProt: E2R1I5 Dog
SwissProt: Q9UFE4 Human
SwissProt: Q9D5Y1 Mouse
Unigene: 712820 Human
Unigene: 474546 Mouse
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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