产品名称
Dynein IC1 Rabbit Polyclonal Antibody
别名
DNAI1; Dynein intermediate chain 1; axonemal; Axonemal dynein intermediate chain 1
蛋白名称
Dynein intermediate chain 1 axonemal
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=27019
Human Swissprot No.
Q9UI46
Human Swissprot Link
http://www.uniprot.org/uniprotkb/Q9UI46/entry
Mouse Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=68922
Mouse Swissprot No.
Q8C0M8
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q8C0M8
Rat Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=500442
Rat Swissprot Link
http://www.uniprot.org/uniprot/Q5XIL8
免疫原
The antiserum was produced against synthesized peptide derived from human DNAI1. AA range:211-260
特异性
Dynein IC1 Polyclonal Antibody detects endogenous levels of Dynein IC1 protein.
稀释度
WB 1:500 - 1:2000. ELISA: 1:20000. Not yet tested in other applications.
宿主
Polyclonal, Rabbit,IgG
背景介绍
This gene encodes a member of the dynein intermediate chain family. The encoded protein is part of the dynein complex in respiratory cilia. 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. Mutations in this gene result in abnormal ciliary ultrastructure and function associated with primary ciliary dyskinesia and Kartagener syndrome. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jul 2013],
组织表达
Expressed in respiratory ciliated cells (at protein level).
细胞定位
Dynein axonemal particle . Cytoplasm, cytoskeleton, cilium axoneme .
信号通路
Huntington's disease;
功能
disease: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).,disease:Defects in DNAI1 are the cause of primary ciliary dyskinesia type 1 (CILD1) [MIM:244400]. 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.,function:Part of the dynein complex of respiratory cilia.,similarity:Belongs to the dynein intermediate chain family.,similarity:Contains 5 WD repeats.,subunit:Consists of at least two heavy chains and a number of intermediate and light chains.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.