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DNAH5 Rabbit pAb (bs-14365R)  
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產(chǎn)品編號 bs-14365R
英文名稱 DNAH5 Rabbit pAb
中文名稱 軸絲動力蛋白5抗體
別    名 axonemal; axonemal; Axonemal beta dynein heavy chain 5; Ciliary dynein heavy chain 5; DNAH 5; Dnah5; DNAHC5; DYH5_HUMAN; Dynein heavy chain 5; Dynein heavy chain 5, axonemal; HL1; KIAA1603.  
研究領(lǐng)域 細胞生物  信號轉(zhuǎn)導(dǎo)  細胞骨架  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human (predicted: Mouse,Rat,Rabbit,Pig,Cow,Horse)
產(chǎn)品應(yīng)用 Flow-Cyt=2ug/test
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 529 kDa
檢測分子量
細胞定位 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human DNAH5: 4001-4200/4624 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 This gene encodes a dynein protein, which is part of a microtubule-associated motor protein complex consisting of heavy, light, and intermediate chains. This protein is an axonemal heavy chain dynein. It functions as a force-generating protein with ATPase activity, whereby the release of ADP is thought to produce the force-producing power stroke. Mutations in this gene cause primary ciliary dyskinesia type 3, as well as Kartagener syndrome, which are both diseases due to ciliary defects. [provided by RefSeq, Oct 2009]

Function:
Force generating protein of respiratory cilia. Produces force towards the minus ends of microtubules. Dynein has ATPase activity; the force-producing power stroke is thought to occur on release of ADP. Required for structural and functional integrity of the cilia of ependymal cells lining the brain ventricles.

Subcellular Location:
Cytoplasm; cytoskeleton; cilium axoneme.

DISEASE:
Defects in DNAH5 are the cause of primary ciliary dyskinesia type 3 (CILD3) [MIM:608644]. CILD3 is an autosomal recessive disorder characterized by axonemal 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 situs inversus, due to dysfunction of monocilia at the embryonic node and randomization of left-right body asymmetry. Primary ciliary dyskinesia associated with situs inversus is referred to as Kartagener syndrome.
Defects in DNAH5 are a cause of Kartagener syndrome (KTGS) [MIM:244400]. KTGS is an autosomal recessive disorder characterized by the association of primary ciliary dyskinesia with situs inversus. Clinical features include recurrent respiratory infections, bronchiectasis, infertility, and lateral transposition of the viscera of the thorax and abdomen. The situs inversus is most often total, although it can be partial in some cases (isolated dextrocardia or isolated transposition of abdominal viscera).

Similarity:
Belongs to the dynein heavy chain family.

SWISS:
Q8TE73

Gene ID:
1767

Database links:

Entrez Gene: 1767 Human

Omim: 603335 Human

SwissProt: Q8TE73 Human

Unigene: 212360 Human



產(chǎn)品圖片
Blank control: A549. Primary Antibody (green line): Rabbit Anti- DNAH5/FITC Conjugated antibody (bs-14365R-FITC) Dilution: 2μg /10^6 cells; Isotype Control Antibody (orange line): Rabbit IgG-FITC . Protocol The cells were fixed with 4% PFA (10min a
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