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Fragile X-related protein family: a double-edged sword in neurodevelopmental disorders and cancer
- Source :
- Crit Rev Biochem Mol Biol
- Publication Year :
- 2020
-
Abstract
- The fragile X-related (FXR) family proteins FMRP, FXR1, and FXR2 are RNA binding proteins that play a critical role in RNA metabolism, neuronal plasticity, and muscle development. These proteins share significant homology in their protein domains, which are functionally and structurally similar to each other. FXR family members are known to play an essential role in causing fragile X mental retardation syndrome (FXS), the most common genetic form of autism spectrum disorder. Recent advances in our understanding of this family of proteins have occurred in tandem with discoveries of great importance to neurological disorders and cancer biology via the identification of their novel RNA and protein targets. Herein, we review the FXR family of proteins as they pertain to FXS, other mental illnesses, and cancer. We emphasize recent findings and analyses that suggest contrasting functions of this protein family in FXS and tumorigenesis based on their expression patterns in human tissues. Finally, we discuss current gaps in our knowledge regarding the FXR protein family and their role in FXS and cancer and suggest future studies to facilitate bench to bedside translation of the findings.
- Subjects :
- congenital, hereditary, and neonatal diseases and abnormalities
Protein family
Protein domain
RNA-binding protein
Biology
medicine.disease_cause
Biochemistry
Homology (biology)
Article
03 medical and health sciences
Fragile X Mental Retardation Protein
Neoplasms
medicine
Humans
Molecular Biology
030304 developmental biology
Genetics
0303 health sciences
030302 biochemistry & molecular biology
RNA
RNA-Binding Proteins
medicine.disease
Autism spectrum disorder
Neurodevelopmental Disorders
Fragile X Syndrome
Mutation
Post-translational protein modification
Carcinogenesis
Subjects
Details
- ISSN :
- 15497798
- Volume :
- 55
- Issue :
- 5
- Database :
- OpenAIRE
- Journal :
- Critical reviews in biochemistry and molecular biology
- Accession number :
- edsair.doi.dedup.....4d3e5aef960ec40f36d58bd43dedf044