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UTP3 small subunit processome component (UTP3)

Target
UTP3
Molecular classification
Other (ribosomal small subunit processome component), RNA-binding protein, Chromatin regulator (functional classification: involved in gene silencing/binding chromatin)
01

Overview

UTP3 small subunit processome component, also known as Sas10 or CRLZ1, is a highly conserved nucleolar RNA-binding protein critical for the biogenesis of the eukaryotic ribosomal small subunit. It is a core component of the small subunit (SSU) processome, ensuring proper maturation of 18S rRNA and coordinating the assembly and nucleolar localization of other SSU processome members such as MPP10, UTP12, UTP13, UTP25, and EMG1[1][2][3]. UTP3 also recruits the RNA exosome enzyme EXOSC10 to the nucleolus, facilitating the processing and degradation of pre-rRNA intermediates[2]. Beyond ribosome biogenesis, UTP3 has roles in gene silencing through chromatin structure regulation[3] and has been linked to cancer chemoresistance via regulation of apoptosis through interaction with the transcription factor c-Myc[1]. Mutations or dysregulation of UTP3 have been associated with a subset of genetic disorders and cancer phenotypes, but it is not currently classified as a therapeutic drug target.

Other names
Sas10CRLZ1
02

Biological functions

Ribosomal small subunit biogenesisRNA bindingChromatin silencingNucleolar localization of other processome proteinsRegulation of protein transportrRNA processing (especially 18S rRNA maturation)
03

Disease associations

Cancer (implicated in chemoresistance in esophageal squamous cell carcinoma through apoptosis regulation)Fanconi anemia (association based on genetic studies)Palmoplantar keratoderma, Bothnian type (association based on genetic studies)Other (potential implication in organogenesis and developmental disorders due to role in ribosome biogenesis)

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