| Radial spoke head protein 9 homolog ELISA Kit|
Radial spoke head protein 9 homolog Recombinant
Radial spoke head protein 9 homolog Antibody
|RSPH9: Probable component of the axonemal radial spoke head. Radial spokes are regularly spaced along cilia, sperm and flagella axonemes. They consist of a thin stalk, which is attached to a subfiber of the outer doublet microtubule, and a bulbous head, which is attached to the stalk and appears to interact with the projections from the central pair of microtubules. Defects in RSPH9 are the cause |
of primary ciliary dyskinesia type 12 (CILD12). 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. Belongs to the flagellar radial spoke RSP9 family. 2 isoforms of the human protein are produced by alternative splicing.
Cellular Component: axoneme
Biological Process: axoneme biogenesis
| Rsph9 ELISA Kit|