Literature DB >> 22633631

Microcephaly-thin corpus callosum syndrome maps to 8q23.2-q24.12.

Ayelet Halevy1, Lina Basel-Vanagaite, Avinoam Shuper, Shlomit Helman, Adi Har-Zahav, Efrat Birk, Idit Maya, Liora Kornreich, Dov Inbar, Gudrun Nürnberg, Peter Nürnberg, Tamar Steinberg, Rachel Straussberg.   

Abstract

Postnatal microcephaly is defined as normal head circumference at birth, which progressively declines to more than 2 standard deviations below the average for the patient's age and sex. We describe four patients from three consanguineous families of Arab Bedouin origin who presented with autosomal recessive inheritance of progressive microcephaly, spasticity, thin corpus callosum, pyramidal signs, and intellectual disability. Homozygosity mapping (Human Mapping NspI 250K arrays, Affymetrix, Santa Clara, CA) placed the disease locus at 8q23.2-q24.12. The candidate region includes 22 known or predicted genes, including RAD21, which is related to the cohesion complex EIF3H, which is involved in translation initiation, and TAF2, which may be involved in intellectual disability. Identification of the causative gene in our reported family will shed light on the pathogenesis of this severe condition.
Copyright © 2012 Elsevier Inc. All rights reserved.

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Year:  2012        PMID: 22633631     DOI: 10.1016/j.pediatrneurol.2012.03.014

Source DB:  PubMed          Journal:  Pediatr Neurol        ISSN: 0887-8994            Impact factor:   3.372


  7 in total

1.  SCRIB and PUF60 are primary drivers of the multisystemic phenotypes of the 8q24.3 copy-number variant.

Authors:  Andrew Dauber; Christelle Golzio; Cécile Guenot; Francine M Jodelka; Maria Kibaek; Susanne Kjaergaard; Bruno Leheup; Danielle Martinet; Malgorzata J M Nowaczyk; Jill A Rosenfeld; Susan Zeesman; Janice Zunich; Jacques S Beckmann; Joel N Hirschhorn; Michelle L Hastings; Sebastien Jacquemont; Nicholas Katsanis
Journal:  Am J Hum Genet       Date:  2013-10-17       Impact factor: 11.025

2.  Langer-Giedion syndrome associated with congenital dural arterio-venous fistula.

Authors:  Severina Leu; Anton Valavanis; Gerasimos Baltsavias
Journal:  Childs Nerv Syst       Date:  2014-10-08       Impact factor: 1.475

Review 3.  Transcription Pause and Escape in Neurodevelopmental Disorders.

Authors:  Kristel N Eigenhuis; Hedda B Somsen; Debbie L C van den Berg
Journal:  Front Neurosci       Date:  2022-05-09       Impact factor: 5.152

4.  Cytoplasmic TAF2-TAF8-TAF10 complex provides evidence for nuclear holo-TFIID assembly from preformed submodules.

Authors:  Simon Trowitzsch; Cristina Viola; Elisabeth Scheer; Sascha Conic; Virginie Chavant; Marjorie Fournier; Gabor Papai; Ima-Obong Ebong; Christiane Schaffitzel; Juan Zou; Matthias Haffke; Juri Rappsilber; Carol V Robinson; Patrick Schultz; Laszlo Tora; Imre Berger
Journal:  Nat Commun       Date:  2015-01-14       Impact factor: 14.919

5.  Early loss of Scribble affects cortical development, interhemispheric connectivity and psychomotor activity.

Authors:  Jerome Ezan; Maité M Moreau; Tamrat M Mamo; Miki Shimbo; Maureen Decroo; Melanie Richter; Ronan Peyroutou; Rivka Rachel; Fadel Tissir; Froylan Calderon de Anda; Nathalie Sans; Mireille Montcouquiol
Journal:  Sci Rep       Date:  2021-04-27       Impact factor: 4.379

6.  A forward genetic screen identifies eukaryotic translation initiation factor 3, subunit H (eIF3h), as an enhancer of variegation in the mouse.

Authors:  Lucia Daxinger; Harald Oey; Anwyn Apedaile; Joanne Sutton; Alyson Ashe; Emma Whitelaw
Journal:  G3 (Bethesda)       Date:  2012-11-01       Impact factor: 3.154

7.  Homozygous TAF8 mutation in a patient with intellectual disability results in undetectable TAF8 protein, but preserved RNA polymerase II transcription.

Authors:  Farrah El-Saafin; Cynthia Curry; Tao Ye; Jean-Marie Garnier; Isabelle Kolb-Cheynel; Matthieu Stierle; Natalie L Downer; Mathew P Dixon; Luc Negroni; Imre Berger; Tim Thomas; Anne K Voss; William Dobyns; Didier Devys; Laszlo Tora
Journal:  Hum Mol Genet       Date:  2018-06-15       Impact factor: 6.150

  7 in total

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