| Primary Identifier | IPR041442 | Type | Domain |
| Short Name | PIH1D1/2/3_CS-like |
| description | This domain is found in animal PIH1 domain-containing protein 1/2/3 (PIH1D1/2/3) and its homologues []. PIH1D1 is part of the HSP90 co-chaperone R2TP complex involved in the assembly process of many molecular machines []. This domain consists of a seven-stranded beta sandwich with the topology of a CS domain, a structural motif also found in Hsp90 co-chaperones such as p23/Sba1 and Sgt1 []. Proteins containing this domain also includes Kintoun from animals and uncharacterised proteins from fungi and plants. Kintoun (also known as ktu or dynein assembly factor 2, axonemal) is a cytoplasmic protein conserved from ciliated unicellular organisms to mammals. It is required for cytoplasmic pre-assembly of axonemal dynein arm complexes before intraflagellar transport loads them for the ciliary compartment. Its homologue in single-celled alga Chlamydomonas is known as PF13. A mutation in Ktu/PF13 has been identified in some patients with primary ciliary dyskinesia (PCD). In the absence of Ktu/PF13, both dynein arms (inner and outer) are missing or defective in the axoneme, leading to a loss of motility []. |
