Product Overview
Description
CLPP-00150569 is recombinant human DNAI1 protein
Applications
ELISA, Western Blot
Protein Length
Full length protein
Nature
Recombinant Protein
Sequence
MIPASAKSPHKQPHKQSISIGRGTRKRDEDSGTEVGEGTDEWAQSKATVRPPDQLELTDAELKEEFTRILTANNPHAPQNIVRYSFKEGTYKPIGFVNQLAVHYTQVGNLIPKDSDEGRRQHYRDELVAGSQESVKVISETGNLEEDEEPKELETEPGSQTDVPAAGAAEKVTEEELMTPKQPKERKLTNQFNFSERASQTCNNPVRDRECQTEPPPRTNFSATANQWEIYDAYVEELEKQEKTKEKEKAKTPVAKKSGKMAMRKLTSMESQTDDLIKLSQAAKIMERMVNQNTYDDIAQDFKYYDDAADEYRDQVGTLLPLWKFQNDKAKRLSVTALCWNPKYRDLFAVGYGSYDFMKQSRGMLLLYSLKNPSFPEYMFSSNSGVMCLDIHVDHPYLVAVGHYDGNVAIYNLKKPHSQPSFCSSAKSGKHSDPVWQVKWQKDDMDQNLNFFSVSSDGRIVSWTLVKRKLVHIDVIKLKVEGSTTEVPEGLQLHQVGCGTAFDFHKEIDYMFLVGTEEGKIYKCSKSYSSQFLDTYDAHNMSVDTVSWNPYHTKVFMSCSSDWTVKIWDHTIKTPMFIYDLNSAVGDVAWAPYSSTVFAAVTTDGKAHIFDLAINKYEAICNQPVAAKKNRLTHVQFNLIHPIIIVGDDRGHIISLKLSPNLRKMPKEKKGQEVQKGPAVEIAKLDKLLNLVREVKIKT
Sequence Similarities
Belongs to the dynein intermediate chain family. Contains 5 WD repeats.
Target Information
Alternative Names
Axonemal dynein intermediate chain 1; Axonemal dynein intermediate chain 2; CILD 1; CILD1; Cytoplasmic dynein 1 intermediate chain 1; Cytoplasmic dynein 1 intermediate chain 2; Cytoplasmic dynein intermediate chain 1; Cytoplasmic dynein intermediate chain 2; DH IC 1; DH IC 2; DIC1; DNAI 1; DNAI 2; DNAI1; DNAI1_HUMAN; DNAI2; DNCI 2; DNCI1; DNCI2; DNCIC 1; DNCIC 2; DNCIC1; DNCIC2; Dynein axonemal intermediate chain 1; Dynein axonemal intermediate polypeptide 1; Dynein axonemal intermediate polypeptide 2; Dynein cytoplasmic intermediate polypeptide 1; Dynein cytoplasmic intermediate polypeptide 2; Dynein intermediate chain 1 axonemal; Dynein intermediate chain 1 cytosolic; Dynein intermediate chain 1, axonemal; Dynein intermediate chain 2 axonemal; Dynein intermediate chain 2 cytosolic; Dynein intermediate chain DNAI1; IC74; ICS; ICS1; Immotile cilia syndrome 1; MGC26204; PCD
Protein Function
Part of the dynein complex of respiratory cilia.
Involvement in Disease
Defects in DNAI1 are the cause of primary ciliary dyskinesia type 1 (CILD1). 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.Defects in DNAI1 are the cause of Kartagener syndrome (KTGS). 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).
Shipping & Handling
Constituents
0.31% Glutathione, 0.79% Tris HCl.
Shipping
Shipped on dry ice.
