Target intelligence / Profile preview

Piercer of microtubule wall 1 protein (PIERCE1)

Target
PIERCE1
Molecular classification
Microtubule inner protein, Other (Structural protein)
01

Overview

Piercer of microtubule wall 1 protein (PIERCE1) is a microtubule inner protein involved in the structural organization of the cilia axoneme, specifically facilitating the attachment of outer dynein arms to dynein-decorated doublet microtubules[4]. The protein plays roles in axoneme assembly, establishment of developmental left/right asymmetry, and sperm motility. PIERCE1 is also predicted to participate in DNA damage response and regulation of gene expression pathways, although these functions are less well characterized. Mutations in PIERCE1 are associated with human ciliopathies such as Meckel syndrome (type 2) and Bardet-Biedl syndrome 3[4]. Do not conflate PIERCE1 with PRC1, the protein regulator of cytokinesis 1. PRC1 is a microtubule-associated protein crucial for mitotic spindle organization and cytokinesis[1][2][3]; while PIERCE1 is primarily linked to ciliary microtubule function[4].

Other names
Pierce1MGC29761RbEST47UPF0691 protein C9orf116p53-induced expression in RB-null cells protein 1p53-induced expression 1 in Rb−/− cellsC9orf116piercer of microtubule wall 1 proteinp53-induced expression 1 in Rba^'/a^' cells
02

Biological functions

Microtubule function in cilia axonemeAttachment of outer dynein arms to doublet microtubules (DMTs) in ciliaAxoneme assemblyEstablishment of left/right asymmetry in developmentFlagellated sperm motilityUpstream of DNA damage response and cellular UV-C response (predicted)Regulation of gene expression (predicted)
03

Disease associations

Meckel syndrome, type 2Bardet-Biedl syndrome 3Other ciliopathies (by association)

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