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DNAI1 antibody, 轴丝中链动力蛋白抗体

 
编 号 PR-4564
产品名称 DNAI1 antibody, 轴丝中链动力蛋白抗体
规 格 0.2ml
价 格 1980元
应 用 WB,IHC,ELISA,ICC,IP,IF
品 牌 Hopebiot

详细信息: 说明书下载

Background:  Involvement in disease: Defects in DNAI1 are the cause of primary ciliary dyskinesia type 1 (CILD1) . CILD1 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 DNAI1 are the 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).

Description: Rabbit polyclonal to DNAI1

Immunogen: KLH conjugated synthetic peptide derived from DNAI1

Specificity:  ·Reacts with Human, Mouse and Rat.

·Isotype: IgG

Application:  ·Western blotting: 1/100-500. Predicted Mol wt: 73 kDa;

·Immunohistochemistry (Paraffin/frozen tissue section): 1/50-200;

·Immunocytochemistry/Immunofluorescence: 1/100;

·Immunoprecipitation: 1/50;

·ELISA: 1/500;

·Optimal working dilutions must be determined by the end user.

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