反应种属
Human;Mouse;Rat;Chicken
蛋白名称
Dynein intermediate chain 2 axonemal
免疫原
The antiserum was produced against synthesized peptide derived from human DNAI2. AA range:71-120
特异性
Dynein IC2 Monoclonal Antibody detects endogenous levels of Dynein IC2 protein.
组成
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
稀释比例
Western Blot: 1/500 - 1/2000
纯化工艺
The antibody was affinity-purified from mouse antiserum by affinity-chromatography using epitope-specific immunogen.
其他名称
DNAI2; Dynein intermediate chain 2; axonemal; Axonemal dynein intermediate chain 2
背景
The protein encoded by this gene belongs to the dynein intermediate chain family, and is part of the dynein complex of respiratory cilia and sperm flagella. Mutations in this gene are associated with primary ciliary dyskinesia type 9. Alternatively spliced transcript variants encoding different isoforms have been noted for this gene. [provided by RefSeq, Mar 2010],
功能
disease:Defects in DNAI2 are the cause of primary ciliary dyskinesia type 9 (CILD9) [MIM:612444]. CILD 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.,sequence caution:Intron retention.,similarity:Belongs to the dynein intermediate chain family.,similarity:Contains 5 WD repeats.,subunit:Consists of at least two heavy chains and a nu