The fragile X mental retardation 1 protein (FMRP) is a master protein regulating the transport and subsequent translation of hundreds of messenger ribonucleic acids (mRNAs) into proteins important for synaptic plasticity that impacts intelligence and emotion (
1). FMRP is missing in males with fragile X syndrome (FXS) and sometimes deficient in carriers and females with FXS. Through evolution the CGG repeat at the 5' end of FMR1 at Xq27.3 has expanded, leading to increased production of FMRP, which has enhanced intelligence and emotional functioning in the evolution of humans.
- Darnell J.C.
- Van Driesche S.J.
- Zhang C.
- Hung K.Y.
- Mele A.
- Fraser C.E.
- et al.
FMRP stalls ribosomal translocation on mRNAs linked to synaptic function and autism.
Cell. 2011; 146: 247-261
To read this article in full you will need to make a payment
Purchase one-time access:Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
One-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:Subscribe to Biological Psychiatry
Already a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
- FMRP stalls ribosomal translocation on mRNAs linked to synaptic function and autism.Cell. 2011; 146: 247-261
- Autism profiles of males with fragile X syndrome.Am J Ment Retard. 2008; 113: 427-438
- A controlled study of longitudinal IQ changes in females and males with fragile X syndrome.Am J Med Genet. 1996; 64: 350-355
- Aberrant frontal lobe maturation in adolescents with fragile X syndrome is related to delayed cognitive maturation.Biol Psychiatry. 2011; 70: 852-858
- Teasing apart the heterogeneity of autism: same behavior, different brains in toddlers with fragile X syndrome and autism.J Neurodevelop Disord. 2009; 1: 81-90
- Cognitive dysfunction and prefrontal synaptic abnormalities in a mouse model of fragile X syndrome.Proc Natl Acad Sci USA. 2011; 108: 2587-2592
- Fragile X: leading the way for targeted treatments in autism.Neurotherapeutics. 2010; 7: 264-274
- Advances in understanding the molecular basis of FXTAS.Hum Mol Genet. 2010; 19: R83-R89
- Decreased fragile X mental retardation protein expression underlies amygdala dysfunction in carriers of the fragile X premutation.Biol Psychiatry. 2011; 70: 859-865
- A review of fragile X premutation disorders: expanding the psychiatric perspective.J Clin Psychiatry. 2009; 70: 852-862
- Autism spectrum disorders and attention-deficit/hyperactivity disorder in boys with the fragile X premutation.J Dev Behav Pediatr. 2006; 27: S137-S144
- Expanded clinical phenotype of women with the FMR1 premutation.Am J Med Genet A. 2008; 146A: 1009-1016
Accepted: September 14, 2011
Received: September 14, 2011
© 2011 Society of Biological Psychiatry. Published by Elsevier Inc. All rights reserved.