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Fragile X mental retardation, autosomal homolog 1
Protein FXR1 PDB 2cpq.png
PDB rendering based on 2cpq.
Available structures
PDB Ortholog search: PDBe, RCSB
Symbols FXR1 ; FXR1P
External IDs OMIM600819 MGI104860 HomoloGene3573 GeneCards: FXR1 Gene
RNA expression pattern
PBB GE FXR1 201636 at tn.png
PBB GE FXR1 201635 s at tn.png
PBB GE FXR1 201637 s at tn.png
More reference expression data
Species Human Mouse
Entrez 8087 14359
Ensembl ENSG00000114416 ENSMUSG00000027680
UniProt P51114 Q61584
RefSeq (mRNA) NM_001013438 NM_001113188
RefSeq (protein) NP_001013456 NP_001106659
Location (UCSC) Chr 3:
180.59 – 180.7 Mb
Chr 3:
34.02 – 34.07 Mb
PubMed search [1] [2]

Fragile X mental retardation syndrome-related protein 1 is a protein that in humans is encoded by the FXR1 gene.[1][2][3]

The protein encoded by this gene is an RNA binding protein that interacts with the functionally similar proteins FMR1 and FXR2. These proteins shuttle between the nucleus and cytoplasm and associate with polyribosomes, predominantly with the 60S ribosomal subunit. Three transcript variants encoding different isoforms have been found for this gene.[3]


FXR1 has been shown to interact with FXR2[4][5] and FMR1.[4][5]


  1. ^ Siomi MC, Siomi H, Sauer WH, Srinivasan S, Nussbaum RL, Dreyfuss G (July 1995). "FXR1, an autosomal homolog of the fragile X mental retardation gene". EMBO J 14 (11): 2401–8. PMC 398353. PMID 7781595. 
  2. ^ Bolivar J, Guelman S, Iglesias C, Ortiz M, Valdivia MM (August 1998). "The fragile-X-related gene FXR1 is a human autoantigen processed during apoptosis". J Biol Chem 273 (27): 17122–7. doi:10.1074/jbc.273.27.17122. PMID 9642279. 
  3. ^ a b "Entrez Gene: FXR1 fragile X mental retardation, autosomal homolog 1". 
  4. ^ a b Siomi, M C; Zhang Y; Siomi H; Dreyfuss G (July 1996). "Specific sequences in the fragile X syndrome protein FMR1 and the FXR proteins mediate their binding to 60S ribosomal subunits and the interactions among them". Mol. Cell. Biol. (UNITED STATES) 16 (7): 3825–32. ISSN 0270-7306. PMC 231379. PMID 8668200. 
  5. ^ a b Zhang, Y; O'Connor J P; Siomi M C; Srinivasan S; Dutra A; Nussbaum R L; Dreyfuss G (November 1995). "The fragile X mental retardation syndrome protein interacts with novel homologs FXR1 and FXR2". EMBO J. (ENGLAND) 14 (21): 5358–66. ISSN 0261-4189. PMC 394645. PMID 7489725. 

Further reading[edit]