Literature DB >> 9259278

Differential expression of FMR1, FXR1 and FXR2 proteins in human brain and testis.

F Tamanini1, R Willemsen, L van Unen, C Bontekoe, H Galjaard, B A Oostra, A T Hoogeveen.   

Abstract

Lack of expression of the fragile X mental retardation protein (FMRP) results in mental retardation and macroorchidism, seen as the major pathological symptoms in fragile X patients. FMRP is a cytoplasmic RNA-binding protein which cosediments with the 60S ribosomal subunit. Recently, two proteins homologous to FMRP were discovered: FXR1 and FXR2. These novel proteins interact with FMRP and with each other and they are also associated with the 60S ribosomal subunit. Here, we studied the expression pattern of the three proteins in brain and testis by immunohistochemistry. In adult brain, FMR1, FXR1 and FXR2 proteins are coexpressed in the cytoplasm of specific differentiated neurons only. However, we observed a different expression pattern in fetal brain as well as in adult and fetal testis, suggesting independent functions for the three proteins in those tissues during embryonic development and adult life.

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Year:  1997        PMID: 9259278     DOI: 10.1093/hmg/6.8.1315

Source DB:  PubMed          Journal:  Hum Mol Genet        ISSN: 0964-6906            Impact factor:   6.150


  60 in total

Review 1.  Fragile X syndrome: the GABAergic system and circuit dysfunction.

Authors:  Scott M Paluszkiewicz; Brandon S Martin; Molly M Huntsman
Journal:  Dev Neurosci       Date:  2011-09-21       Impact factor: 2.984

2.  FXR1P but not FMRP regulates the levels of mammalian brain-specific microRNA-9 and microRNA-124.

Authors:  Xia-Lian Xu; Ruiting Zong; Zhaodong Li; Md Helal Uddin Biswas; Zhe Fang; David L Nelson; Fen-Biao Gao
Journal:  J Neurosci       Date:  2011-09-28       Impact factor: 6.167

3.  Characterisation of Fmrp in zebrafish: evolutionary dynamics of the fmr1 gene.

Authors:  Sandra van 't Padje; Bart Engels; Lau Blonden; Lies-Anne Severijnen; Frans Verheijen; Ben A Oostra; Rob Willemsen
Journal:  Dev Genes Evol       Date:  2005-01-27       Impact factor: 0.900

4.  Transport of fragile X mental retardation protein via granules in neurites of PC12 cells.

Authors:  Yolanda De Diego Otero; Lies-Anne Severijnen; Gert van Cappellen; Mariëtte Schrier; Ben Oostra; Rob Willemsen
Journal:  Mol Cell Biol       Date:  2002-12       Impact factor: 4.272

5.  The Longitudinal Effects of Parenting on Adaptive Behavior in Children with Fragile X Syndrome.

Authors:  Steven F Warren; Nancy Brady; Kandace K Fleming; Laura J Hahn
Journal:  J Autism Dev Disord       Date:  2017-03

Review 6.  FMR1: a gene with three faces.

Authors:  Ben A Oostra; Rob Willemsen
Journal:  Biochim Biophys Acta       Date:  2009-02-21

7.  Prepulse inhibition in fragile X syndrome: feasibility, reliability, and implications for treatment.

Authors:  David Hessl; Elizabeth Berry-Kravis; Lisa Cordeiro; Jennifer Yuhas; Edward M Ornitz; Aaron Campbell; Elizabeth Chruscinski; Crystal Hervey; James M Long; Randi J Hagerman
Journal:  Am J Med Genet B Neuropsychiatr Genet       Date:  2009-06-05       Impact factor: 3.568

8.  Discrimination of common and unique RNA-binding activities among Fragile X mental retardation protein paralogs.

Authors:  Jennifer C Darnell; Claire E Fraser; Olga Mostovetsky; Robert B Darnell
Journal:  Hum Mol Genet       Date:  2009-06-01       Impact factor: 6.150

9.  Generation and characterization of FMR1 knockout zebrafish.

Authors:  Marjo J den Broeder; Herma van der Linde; Judith R Brouwer; Ben A Oostra; Rob Willemsen; René F Ketting
Journal:  PLoS One       Date:  2009-11-19       Impact factor: 3.240

10.  A solution to limitations of cognitive testing in children with intellectual disabilities: the case of fragile X syndrome.

Authors:  David Hessl; Danh V Nguyen; Cherie Green; Alyssa Chavez; Flora Tassone; Randi J Hagerman; Damla Senturk; Andrea Schneider; Amy Lightbody; Allan L Reiss; Scott Hall
Journal:  J Neurodev Disord       Date:  2009-03       Impact factor: 4.025

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