Literature DB >> 31191205

1q42.12q42.2 Deletion in a Child with Midline Defects and Hypoplasia of the Corpus Callosum.

Akella Radha Rama Devi1, Aparna Ganapathy2, Ashraf U Mannan2, Shefali Sabharanjak2, Shaik M Naushad3.   

Abstract

Chromosome 1q42.12q42.2 deletions are documented as "disease causing" and show overlapping phenotypes depending on the genes involved in the deletion. In this report, we detected a 5.8-Mb deletion encompassing the chromosome 1q42.12q42.2 region in a 4-year-old boy with hypoplastic corpus callosum, epilepsy, developmental delay, microcephaly, cataract, cleft palate, and skeletal changes. The deletion was de novo. Genotype-phenotype correlations suggest that the major features of 1q42.12q42.2 microdeletion were attributed to the genes with a high probability of loss-of-function intolerance score in this deletion, namely LBR, ENAH, ACBD3, LIN9, ITPKB, CDC42BPA, ARF1, TAF5L, GALNT2, SPRTN, and EGLN1 along with GNPAT.

Entities:  

Keywords:  1q42.12q42.2 deletion; GNPAT; LBR; pLI score

Year:  2019        PMID: 31191205      PMCID: PMC6528072          DOI: 10.1159/000496079

Source DB:  PubMed          Journal:  Mol Syndromol        ISSN: 1661-8769


  21 in total

1.  Primer3 on the WWW for general users and for biologist programmers.

Authors:  S Rozen; H Skaletsky
Journal:  Methods Mol Biol       Date:  2000

2.  Mena is required for neurulation and commissure formation.

Authors:  L M Lanier; M A Gates; W Witke; A S Menzies; A M Wehman; J D Macklis; D Kwiatkowski; P Soriano; F B Gertler
Journal:  Neuron       Date:  1999-02       Impact factor: 17.173

3.  New cases and refinement of the critical region in the 1q41q42 microdeletion syndrome.

Authors:  Jill A Rosenfeld; Yves Lacassie; Dima El-Khechen; Luis F Escobar; James Reggin; Carolyn Heuer; Emily Chen; Lauren S Jenkins; A Thomas Collins; Samuel Zinner; Melanie Babcock; Bernice Morrow; Roger A Schultz; Beth S Torchia; Blake C Ballif; Karen D Tsuchiya; Lisa G Shaffer
Journal:  Eur J Med Genet       Date:  2010-10-15       Impact factor: 2.708

4.  Interstitial deletion 1q42 in a patient with agenesis of corpus callosum: Phenotype-genotype comparison to the 1q41q42 microdeletion suggests a contiguous 1q4 syndrome.

Authors:  Isabel Filges; Benno Röthlisberger; Nemya Boesch; Peter Weber; Friedel Wenzel; Andreas R Huber; Karl Heinimann; Peter Miny
Journal:  Am J Med Genet A       Date:  2010-04       Impact factor: 2.802

5.  Findings from aCGH in patients with congenital diaphragmatic hernia (CDH): a possible locus for Fryns syndrome.

Authors:  S Kantarci; D Casavant; C Lee; V Kimonis; B R Pober; C Prada; M Russell; J Byrne; L Wilkins Haug; R Jennings; S Manning; T K Boyd; J P Fryns; L B Holmes; P K Donahoe
Journal:  Am J Med Genet A       Date:  2006-01-01       Impact factor: 2.802

6.  Lin9, a subunit of the mammalian DREAM complex, is essential for embryonic development, for survival of adult mice, and for tumor suppression.

Authors:  Nina Reichert; Sebastian Wurster; Tanja Ulrich; Kathrin Schmitt; Stefanie Hauser; Leona Probst; Rudolf Götz; Fatih Ceteci; Roland Moll; Ulf Rapp; Stefan Gaubatz
Journal:  Mol Cell Biol       Date:  2010-04-19       Impact factor: 4.272

7.  Dosage effect of zero to three functional LBR-genes in vivo and in vitro.

Authors:  Sophia Gravemann; Nele Schnipper; Hannes Meyer; Amparo Vaya; Malgorzata Jm Nowaczyk; Anna Rajab; Wolf-Karsten Hofmann; Bastian Salewsky; Holger Tönnies; Heidemarie Neitzel; Hans H Stassen; Karl Sperling; Katrin Hoffmann
Journal:  Nucleus       Date:  2010-01-03       Impact factor: 4.197

8.  Functional characterization of novel mutations in GNPAT and AGPS, causing rhizomelic chondrodysplasia punctata (RCDP) types 2 and 3.

Authors:  Brandon Itzkovitz; Sarn Jiralerspong; Graeme Nimmo; Melissa Loscalzo; Dafne D G Horovitz; Ann Snowden; Ann Moser; Steve Steinberg; Nancy Braverman
Journal:  Hum Mutat       Date:  2011-10-31       Impact factor: 4.878

Review 9.  The discovery of microdeletion syndromes in the post-genomic era: review of the methodology and characterization of a new 1q41q42 microdeletion syndrome.

Authors:  Lisa G Shaffer; Aaron Theisen; Bassem A Bejjani; Blake C Ballif; Arthur S Aylsworth; Cynthia Lim; Marie McDonald; Jay W Ellison; Dana Kostiner; Sulagna Saitta; Tamim Shaikh
Journal:  Genet Med       Date:  2007-09       Impact factor: 8.822

10.  RNAi-mediated silencing of ABCD3 gene expression in rat C6 glial cells: a model system to study PMP70 function.

Authors:  Rita Di Benedetto; Michela Alessandra Denti; Serafina Salvati; Massimo Sanchez; Lucilla Attorri; Giulia David; Antonella Di Biase
Journal:  Neurochem Int       Date:  2007-11-29       Impact factor: 3.921

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