The roles of NOP56 in cancer and SCA36

Pathol Oncol Res. 2023 Jan 19:29:1610884. doi: 10.3389/pore.2023.1610884. eCollection 2023.

Abstract

NOP56 is a highly conserved nucleolar protein. Amplification of the intron GGCCTG hexanucleotide repeat sequence of the NOP56 gene results in spinal cerebellar ataxia type 36 (SCA36). NOP56 contains an N-terminal domain, a coiled-coil domain, and a C-terminal domain. Nucleolar protein NOP56 is significantly abnormally expressed in a number of malignant tumors, and its mechanism is different in different tumors, but its regulatory mechanism in most tumors has not been fully explored. NOP56 promotes tumorigenesis in some cancers and inhibits tumorigenesis in others. In addition, NOP56 is associated with methylation in some tumors, suggesting that NOP56 has the potential to become a tumor-specific marker. This review focuses on the structure, function, related signaling pathways, and role of NOP56 in the progression of various malignancies, and discusses the progression of NOP56 in neurodegenerative and other diseases.

Keywords: NOP56; SCA36; box C/D RNP; malignant tumor; methylation.

Publication types

  • Review

MeSH terms

  • Carcinogenesis
  • Cerebellar Ataxia*
  • Humans
  • Neoplasms* / genetics
  • Nuclear Proteins* / genetics
  • Nuclear Proteins* / metabolism
  • Spinocerebellar Ataxias* / genetics
  • Spinocerebellar Ataxias* / pathology

Substances

  • Nuclear Proteins
  • NOP56 protein, human

Grants and funding

The study was supported by the Key Research and Development Program of Jiangxi Province Grant/Award number: 20181BBG70019 and the National Natural Science Foundation of JH Grant/Award number: 82060445.