Literature DB >> 31927143

Fragile X syndrome and associated disorders: Clinical aspects and pathology.

Maria Jimena Salcedo-Arellano1, Brett Dufour2, Yingratana McLennan3, Veronica Martinez-Cerdeno4, Randi Hagerman5.   

Abstract

This review aims to assemble many years of research and clinical experience in the fields of neurodevelopment and neuroscience to present an up-to-date understanding of the clinical presentation, molecular and brain pathology associated with Fragile X syndrome, a neurodevelopmental condition that develops with the full mutation of the FMR1 gene, located in the q27.3 loci of the X chromosome, and Fragile X-associated tremor/ataxia syndrome a neurodegenerative disease experienced by aging premutation carriers of the FMR1 gene. It is important to understand that these two syndromes have a very distinct clinical and pathological presentation while sharing the same origin: the mutation of the FMR1 gene; revealing the complexity of expansion genetics.
Copyright © 2020 The Authors. Published by Elsevier Inc. All rights reserved.

Entities:  

Keywords:  FMR1 gene; FXTAS; Fragile X syndrome; Fragile X-associated tremor/ataxia syndrome; Neuropathology

Mesh:

Substances:

Year:  2020        PMID: 31927143      PMCID: PMC7027994          DOI: 10.1016/j.nbd.2020.104740

Source DB:  PubMed          Journal:  Neurobiol Dis        ISSN: 0969-9961            Impact factor:   7.046


  123 in total

1.  Analysis of neocortex in three males with the fragile X syndrome.

Authors:  V J Hinton; W T Brown; K Wisniewski; R D Rudelli
Journal:  Am J Med Genet       Date:  1991-12-01

2.  Dysregulated ADAM10-Mediated Processing of APP during a Critical Time Window Leads to Synaptic Deficits in Fragile X Syndrome.

Authors:  Emanuela Pasciuto; Tariq Ahmed; Tina Wahle; Fabrizio Gardoni; Laura D'Andrea; Laura Pacini; Sébastien Jacquemont; Flora Tassone; Detlef Balschun; Carlos G Dotti; Zsuzsanna Callaerts-Vegh; Rudi D'Hooge; Ulrike C Müller; Monica Di Luca; Bart De Strooper; Claudia Bagni
Journal:  Neuron       Date:  2015-07-15       Impact factor: 17.173

3.  Immunocytochemical and biochemical characterization of FMRP, FXR1P, and FXR2P in the mouse.

Authors:  C E Bakker; Y de Diego Otero; C Bontekoe; P Raghoe; T Luteijn; A T Hoogeveen; B A Oostra; R Willemsen
Journal:  Exp Cell Res       Date:  2000-07-10       Impact factor: 3.905

4.  Abnormal trajectories in cerebellum and brainstem volumes in carriers of the fragile X premutation.

Authors:  Jun Yi Wang; David Hessl; Randi J Hagerman; Tony J Simon; Flora Tassone; Emilio Ferrer; Susan M Rivera
Journal:  Neurobiol Aging       Date:  2017-03-18       Impact factor: 4.673

Review 5.  Premature ovarian failure in the fragile X syndrome.

Authors:  S L Sherman
Journal:  Am J Med Genet       Date:  2000

Review 6.  The translation of translational control by FMRP: therapeutic targets for FXS.

Authors:  Jennifer C Darnell; Eric Klann
Journal:  Nat Neurosci       Date:  2013-04-14       Impact factor: 24.884

7.  Alcohol use dependence in fragile X syndrome.

Authors:  María J Salcedo-Arellano; Reymundo Lozano; Flora Tassone; Randi J Hagerman; Wilmar Saldarriaga
Journal:  Intractable Rare Dis Res       Date:  2016-08

8.  Neuropathologic features in the hippocampus and cerebellum of three older men with fragile X syndrome.

Authors:  Claudia M Greco; Celestine S Navarro; Michael R Hunsaker; Izumi Maezawa; John F Shuler; Flora Tassone; Mary Delany; Jacky W Au; Robert F Berman; Lee-Way Jin; Cynthia Schumann; Paul J Hagerman; Randi J Hagerman
Journal:  Mol Autism       Date:  2011-02-08       Impact factor: 7.509

9.  Astroglial-targeted expression of the fragile X CGG repeat premutation in mice yields RAN translation, motor deficits and possible evidence for cell-to-cell propagation of FXTAS pathology.

Authors:  H Jürgen Wenzel; Karl D Murray; Saif N Haify; Michael R Hunsaker; Jared J Schwartzer; Kyoungmi Kim; Albert R La Spada; Bryce L Sopher; Paul J Hagerman; Christopher Raske; Lies-Anne W F M Severijnen; Rob Willemsen; Renate K Hukema; Robert F Berman
Journal:  Acta Neuropathol Commun       Date:  2019-02-26       Impact factor: 7.801

10.  Fragile X Mental Retardation Protein is Involved in Protein Synthesis-Dependent Collapse of Growth Cones Induced by Semaphorin-3A.

Authors:  Chanxia Li; Gary J Bassell; Yukio Sasaki
Journal:  Front Neural Circuits       Date:  2009-09-15       Impact factor: 3.492

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  21 in total

1.  FMRP regulates GABAA receptor channel activity to control signal integration in hippocampal granule cells.

Authors:  Pan-Yue Deng; Ajeet Kumar; Valeria Cavalli; Vitaly A Klyachko
Journal:  Cell Rep       Date:  2022-05-17       Impact factor: 9.995

2.  Deletion of Fmr1 in parvalbumin-expressing neurons results in dysregulated translation and selective behavioral deficits associated with fragile X syndrome.

Authors:  Magdalena Kalinowska; Mathijs B van der Lei; Michael Kitiashvili; Maggie Mamcarz; Mauricio M Oliveira; Francesco Longo; Eric Klann
Journal:  Mol Autism       Date:  2022-06-29       Impact factor: 6.476

3.  Dystonia in a Female Fragile X Premutation Carrier.

Authors:  Victoria Ros-Castelló; Anna Latorre; Juan Álvarez-Linera; Juan C Martinez-Castrillo; Kailash P Bhatia; Isabel Pareés
Journal:  Mov Disord Clin Pract       Date:  2021-05-22

Review 4.  Neurodevelopmental Disorders Commonly Presenting with Sleep Disturbances.

Authors:  Althea Robinson Shelton; Beth Malow
Journal:  Neurotherapeutics       Date:  2021-01-05       Impact factor: 7.620

5.  Differentially Expressed Circular RNAs in Peripheral Blood Mononuclear Cells of Patients with Parkinson's Disease.

Authors:  Stylianos Ravanidis; Anastasia Bougea; Dimitra Karampatsi; Nikolaos Papagiannakis; Matina Maniati; Leonidas Stefanis; Epaminondas Doxakis
Journal:  Mov Disord       Date:  2021-01-12       Impact factor: 10.338

Review 6.  Fragile X-Associated Tremor/Ataxia Syndrome (FXTAS): Pathophysiology and Clinical Implications.

Authors:  Ana Maria Cabal-Herrera; Nattaporn Tassanakijpanich; Maria Jimena Salcedo-Arellano; Randi J Hagerman
Journal:  Int J Mol Sci       Date:  2020-06-20       Impact factor: 5.923

Review 7.  Molecules, Mechanisms, and Disorders of Self-Domestication: Keys for Understanding Emotional and Social Communication from an Evolutionary Perspective.

Authors:  Goran Šimić; Vana Vukić; Janja Kopić; Željka Krsnik; Patrick R Hof
Journal:  Biomolecules       Date:  2020-12-22

8.  GABAA Alpha 2,3 Modulation Improves Select Phenotypes in a Mouse Model of Fragile X Syndrome.

Authors:  Tori L Schaefer; Amy A Ashworth; Durgesh Tiwari; Madison P Tomasek; Emma V Parkins; Angela R White; Andrew Snider; Matthew H Davenport; Lindsay M Grainger; Robert A Becker; Chandler K Robinson; Rishav Mukherjee; Michael T Williams; Jay R Gibson; Kimberly M Huber; Christina Gross; Craig A Erickson
Journal:  Front Psychiatry       Date:  2021-05-21       Impact factor: 4.157

9.  Characterization of the Cerebrospinal Fluid Proteome in Patients with Fragile X-Associated Tremor/Ataxia Syndrome.

Authors:  Diana A Abbasi; Thu T A Nguyen; Deborah A Hall; Erin Robertson-Dick; Elizabeth Berry-Kravis; Stephanie M Cologna
Journal:  Cerebellum       Date:  2021-05-27       Impact factor: 3.847

10.  The Fuchs corneal dystrophy-associated CTG repeat expansion in the TCF4 gene affects transcription from its alternative promoters.

Authors:  Alex Sirp; Kristian Leite; Jürgen Tuvikene; Kaja Nurm; Mari Sepp; Tõnis Timmusk
Journal:  Sci Rep       Date:  2020-10-28       Impact factor: 4.379

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