HEAT repeat-containing protein 1 (HEATR1)

HEAT repeat-containing protein 1 (HEATR1) is a human nucleolar protein that serves as the structural and functional homolog of yeast U3 small nucleolar RNA-associated protein 10 (UTP10)[1][2][6]. It plays a crucial role in the processing of pre-18S ribosomal RNA, functioning within the small subunit (SSU) processome in association with U3 snoRNA[1][3][6]. HEATR1 participates in both pre-rRNA transcription and processing, earning the classification as a "transcription-UTP" (t-UTP) protein, and acts as an integrative hub for ribosomal RNA synthesis and maturation[1]. Depletion of HEATR1 results in a block of RNA polymerase I-mediated transcription, nucleolar structural disruption, and activation of a ribosome biogenesis stress checkpoint involving the RPL5/RPL11-MDM2-p53 pathway, leading to p53 stabilization and cell cycle arrest[1]. Increased HEATR1 expression is observed in various human cancers and may be linked to malignant transformation through effects on ribosome biogenesis. It is not known to be the direct target of any approved or investigational drugs, nor are there known mechanism-of-action or safety-concern annotations relating to therapeutic targeting[1][2][5][6].

Other names

HEATR1BAP28UTP10FLJ10359Protein BAP28U3 small nucleolar RNA-associated protein 10 homologsmall subunit (SSU) processome component homolog (yeast)