轴丝中链动力蛋白抗体
规格：1尘驳/1尘濒
英文名： DNAI1 + 2
别名： DNAI1; DNAI1 / 2; DNAI1/2; DNAI 1/2; DNAI 1 + 2; Axonemal dynein intermediate chain 1; Axonemal dynein intermediate chain 2; CILD 1; CILD1; DNAI 1; DNAI 2; DNAI1_HUMAN; Dynein axonemal intermediate ch
分子量： 79kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human DNAI1/2
交叉反应：Human, Mouse, Rat, Dog, Cow, Horse, Rabbit, Sheep,
细胞定位：细胞浆
轴丝中链动力蛋白抗体产物介绍：background: The inner- and outer-arm dyneins, which bridge between the doublet microtubules in axonemes, are the force-generating proteins responsible for the sliding movement in axonemes. The intermediate and light chains, thought to form the base of the dynein arm, help mediate attachment and may also participate in regulating dynein activity. This gene encodes an intermediate chain dynein, belonging to the large family of motor proteins. Mutations in this gene result in abnormal ciliary ultrastructure and function associated with primary ciliary dyskinesia (PCD) and Kartagener syndrome. [provided by RefSeq, Jul 2008]. Function: Part of the dynein complex of respiratory cilia. Subunit: Consists of at least two heavy chains and a number of intermediate and light chains. Subcellular Location: Cytoplasm, cytoskeleton, cilium axoneme. DISEASE: Defects in DNAI1 are the cause of primary ciliary dyskinesia type 1 (CILD1) [MIM:244400]. CILD1 is an autosomal 轴丝中链动力蛋白抗体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 轴丝中链动力蛋白抗体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 intermediate chain family. Contains 5 WD repeats. Database links: Entrez Gene: 27019 Human Entrez Gene: 68922 Mouse Entrez Gene: 500442 Rat Omim: 603772 Human SwissProt: Q9UI46 Human SwissProt: Q8C0M8 Mouse SwissProt: Q5XIL8 Rat Unigene: 112667 Human Unigene: 79127 Mouse Unigene: 93980 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
轴丝中链动力蛋白抗体产物应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：神经生物学 信号转导 细胞粘附分子 细胞骨架
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid