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DNAI1/Dynein intermediate chain 1轴丝中链动力蛋白抗体
点击次数:239发布时间:2012/12/5 13:33:03
更新日期:2024/9/5 14:43:01
所 在 地:中国大陆
产品型号:BY-11024R
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货号:BY-11024R
英文名称:Anti-DNAI1/Dynein intermediate chain 1
中文名称:轴丝中链动力蛋白抗体
其他名称:名Axonemal dynein intermediate chain 1;Axonemal dynein intermediate chain 2;CILD 1;CILD1;Cytoplasmic dynein 1 intermediate chain 1;Cytoplasmic dynein 1 intermediate chain 2;Cytoplasmic dynein intermediate chain 1;Cytoplasmic dynein intermediate chain 2;DH IC 1;DH IC 2;DNAI 1;DNAI 2;DNAI1;DNAI1_HUMAN;DNAI2;DNCI 2;DNCI1;DNCI2;DNCIC 1;DNCIC 2;DNCIC1;DNCIC2;DYNC1I1;DYNC1I2;Dynein axonemal intermediate chain 1;Dynein axonemal intermediate polypeptide 1;Dynein axonemal intermediate polypeptide 2;Dynein cytoplasmic intermediate polypeptide 1;Dynein cytoplasmic intermediate polypeptide 2;Dynein intermediate chain 1 axonemal;Dynein intermediate chain 1 cytosolic;Dynein intermediate chain 1, axonemal;Dynein intermediate chain 2 axonemal;Dynein intermediate chain 2 cytosolic;Dynein intermediate chain DNAI1;ICS;MGC26204;PCD.
抗体来源:Rabbit
克隆类型:polyclonal
蛋白分子量:predicted molecular weight: 73kDa
纯化方法:affinity purified by Protein A
交叉反应:hu, rat, cow
产品介绍: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).Function : Part of the dynein complex of respiratory cilia.Subcellular Location : Cytoplasm > cytoskeleton > cilium axoneme.