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TENT5/FAM46: An Enigmatic Family of Secretory Tuners.
Daniel Lacidogna1, Sara Pennacchio1, Enrico Milan1,2
1Division of Genetics and Cell Biology, IRCCS San Raffaele Scientific Institute, Milano, Italy.
Traffic (Copenhagen, Denmark)
|May 23, 2025
Summary
The TENT5 family of proteins fine-tunes the expression of secretory proteins, impacting cell functions. Mutations in TENT5 genes are linked to diseases including osteogenesis imperfecta, multiple myeloma, and infertility.
Area of Science:
- Biochemistry
- Molecular Biology
- Cell Biology
Background:
- The human TENT5 family (TENT5A-D) comprises noncanonical poly(A)polymerases.
- These proteins selectively stabilize mRNAs for endoplasmic reticulum (ER)-imported proteins.
- TENT5 proteins are crucial for secretory cargo expression and the secretory apparatus.
Purpose of the Study:
- To comprehensively review the cellular functions of TENT5 proteins.
- To analyze their roles in physiological and pathological conditions.
- To highlight proposed molecular mechanisms and identify open questions.
Main Methods:
- Literature review of studies on TENT5 family members.
- Analysis of TENT5 involvement in various cellular processes.
- Examination of TENT5 interactions with other proteins, such as FNDC3.
Main Results:
- TENT5A mutations cause osteogenesis imperfecta by impairing collagen deposition.
- TENT5C alterations are linked to multiple myeloma.
- TENT5D mutations are associated with male infertility.
- TENT5 proteins' specificity is proposed to stem from their ER membrane localization via FNDC3 interactions.
Conclusions:
- TENT5 proteins are key regulators of secretory cell function.
- Dysregulation of TENT5 is implicated in diverse diseases, including cancer and bone disorders.
- Further research is needed to fully elucidate TENT5 functions and therapeutic potential.
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