Molecular weight (DA)
79kD
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.
Dilution rate
Western Blot: 1/500 - 1/2000. ELISA: 1/20000. Not yet tested in other applications.
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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
Protein name
Dynein intermediate chain 1 axonemal
Other name
DNAI1; Dynein intermediate chain 1; axonemal; Axonemal dynein intermediate chain 1
Fields
>>Amyotrophic lateral sclerosis;>>Huntington disease;>>Pathways of neurodegeneration - multiple diseases
Human protein sequence Database
Q9UI46
Mouse protein sequence database
Q8C0M8
Rat protein sequence database
Q5XIL8
Cellular localization
Dynein axonemal particle . Cytoplasm, cytoskeleton, cilium axoneme .
Tissue expression
Expressed in respiratory ciliated cells (at protein level).
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