Fragile X Mental Retardation Protein (FMRP), and its autosomal paralogues,
Fragile X mental Retardation syndrome-related protein 1 and 2 (FXR1 and FXR2),
comprise a family of RNA-binding proteins. It is thought that the FRMP family
of proteins contributes to the regulation of protein synthesis at sites where
mRNAs are locally translated in response to stimuli. These proteins are highly
similar to one another and also retain highly conserved domain architecture.
The two ribonucleoprotein K homology (KH) domains (see <PDOC50084>) and the
cluster of arginine and glycine residues that constitute the RGG box, comprise
a large region that is important for RNA binding and polyribosome association.
In addition, two Agenet-like domains exist in tandem within the N-terminal
regions of FMRP family proteins. The Agenet-like domain belongs to the "Royal"
domain superfamilly which contains also the Tudor (see <PDOC50304>), chromo
(see <PDOC00517>), MBT (see <PDOC51079>), PWWP (see <PDOC50812>) and plant
Agenet domains. Biochemical analysis of the tandem Agenet-like domains reveals
their ability to preferentially recognize trimethylated peptides in a
sequence-specific manner [1,2,3,4].
The Agenet-like domain folds into a bent four-stranded antiparallel β sheet
with a fifth strand closing the cavity of the sheet, similar to a thumb
accross a semiclosed hand (see <PDB:2BKD>) [3,4].
The profile we developed covers the entire Agenet-like domain.
March 2012 / First entry.
PROSITE method (with tools and information) covered by this documentation:
Maurer-Stroh S. Dickens N.J. Hughes-Davies L. Kouzarides T. Eisenhaber F. Ponting C.P.
The Tudor domain 'Royal Family': Tudor, plant Agenet, Chromo, PWWP and MBT domains.
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