Target：Dynein IC1

Fields：Amyotrophic lateral sclerosis;Huntington disease;Pathways of neurodegeneration - multiple diseases

Gene Name：DNAI1

Protein Name：Dynein intermediate chain 1 axonemal

Human Gene Id：27019

Human Swiss Prot No：Q9UI46

Mouse Gene Id：68922

Mouse Swiss Prot No：Q8C0M8

Rat Gene Id：500442

Rat Swiss Prot No：Q5XIL8

Immunogen：The antiserum was produced against synthesized peptide derived from human DNAI1. AA range:211-260

Specificity：Dynein IC1 Polyclonal Antibody detects endogenous levels of Dynein IC1 protein.

Formulation：Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Source：Polyclonal, Rabbit,IgG

Dilution：WB 1:500 - 1:2000. ELISA: 1:20000. Not yet tested in other applications.

Purification：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration：1 mg/ml

Storage Stability：-15°C to -25°C/1 year(Do not lower than -25°C)

Other Name：DNAI1;Dynein intermediate chain 1; axonemal;Axonemal dynein intermediate chain 1

Observed Band(KD)：79kD

Background： 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],

Function：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

Subcellular Location：Dynein axonemal particle . Cytoplasm, cytoskeleton, cilium axoneme .

Expression：Expressed in respiratory ciliated cells (at protein level).