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<front>
<journal-meta>
<journal-id journal-id-type="issn">1043-3155</journal-id>
<journal-id journal-id-type="nlm-ta">Pediatr Neurol Briefs</journal-id>
<journal-id journal-id-type="pmc">pedneurbriefs</journal-id>
<journal-id journal-id-type="iso-abbrev">Pediatr Neurol Briefs</journal-id>
<journal-title-group>
<journal-title>Pediatric Neurology Briefs</journal-title>
<abbrev-journal-title>Pediatr Neurol Briefs</abbrev-journal-title>
</journal-title-group>
<issn pub-type="epub">2166-6482</issn>
<issn pub-type="ppub">1043-3155</issn>
<issn-l>2166-3155</issn-l>
<publisher>
<publisher-name>Pediatric Neurology Briefs Publishers</publisher-name>
<publisher-loc>Chicago, IL, USA</publisher-loc>
</publisher>
</journal-meta>
<article-meta>
<article-id pub-id-type="publisher-id">PNB-2014-28-5-3</article-id>
<article-id pub-id-type="doi">10.15844/pedneurbriefs-28-5-3</article-id>
<article-categories>
<subj-group subj-group-type="heading">
<subject>Febrile Seizures</subject>
</subj-group>
<subj-group subj-group-type="Discipline-v2">
<subject>Neurology</subject>
<subject>Pediatrics</subject>
<subject>Nervous System Diseases</subject>
<subject>Child Development</subject>
<subject>Brain Diseases</subject>
<subject>Neurosurgery</subject>
<subject>Child</subject>
<subject>Infant</subject>
</subj-group>
</article-categories>
<title-group>
<article-title>Genetic Factor in Etiology of Febrile Seizures</article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author" corresp="yes">
<contrib-id contrib-id-type="orcid">http://orcid.org/0000-0002-0173-7931</contrib-id>
<name>
<surname>Millichap</surname>
<given-names>J. Gordon</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="AF0001">1</xref>
<xref ref-type="aff" rid="AF0002">2</xref>
<xref ref-type="corresp" rid="cor1">&#x002A;</xref>
</contrib>
<contrib contrib-type="author">
<contrib-id contrib-id-type="orcid">http://orcid.org/0000-0002-0798-0131</contrib-id>
<name>
<surname>Millichap</surname>
<given-names>John J.</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="AF0001">1</xref>
<xref ref-type="aff" rid="AF0002">2</xref>
</contrib>
</contrib-group>
<aff id="AF0001">
<label>1</label>Division of Neurology, Ann &#x0026; Robert H. Lurie Children&#x0027;s Hospital of Chicago, Chicago, IL</aff>
<aff id="AF0002">
<label>2</label>Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL</aff>
<author-notes>
<corresp id="cor1">
<label>&#x002A;</label>Correspondence: Dr. J. Gordon Millichap, E-mail: <email xlink:href="jgmillichap@northwestern.edu">jgmillichap@northwestern.edu</email>
</corresp>
</author-notes>
<pub-date date-type="pub" publication-format="print">
<month>05</month>
<year>2014</year>
</pub-date>
<pub-date date-type="pub" publication-format="electronic">
<day>31</day>
<month>10</month>
<year>2015</year>
</pub-date>
<volume>28</volume>
<issue>5</issue>
<fpage>35</fpage>
<lpage>35</lpage>
<permissions>
<copyright-statement>Copyright: &#x00A9; 2014 The Author(s)</copyright-statement>
<copyright-year>2014</copyright-year>
<license license-type="open-access" xlink:href="http://creativecommons.org/licenses/by/4.0/">
<license-p>This work is licensed under the <uri xlink:href="http://creativecommons.org/licenses/by/4.0/">Creative Commons Attribution 4.0 International License</uri>, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.</license-p>
</license>
</permissions>
<related-article id="R1" related-article-type="commentary-article" ext-link-type="doi" xlink:href="10.1016/j.pediatrneurol.2014.01.002" vol="50" page="353">
<article-title>The GABAa receptor g2 subunit (R43Q) mutation in febrile seizures</article-title>
</related-article>
<abstract abstract-type="web-summary" specific-use="electronic-only">
<p>Investigators from Istanbul, Turkey, studied R43Q mutations of the gamma-aminobutyric acid A receptor (GABRG2) gene, located on the long arm of chromosome 5, in 44 children with febrile seizure (FS) and 49 without.</p>
</abstract>
<kwd-group>
<kwd>Gamma-Aminobutyric Acid</kwd>
<kwd>Febrile Seizure</kwd>
<kwd>Ligand-Gated</kwd>
</kwd-group>
</article-meta>
</front>
<body>
<p>Investigators from Istanbul, Turkey, studied R43Q mutations of the gamma-aminobutyric acid A receptor (GABRG2) gene, located on the long arm of chromosome 5, in 44 children with febrile seizure (FS) and 49 without. FSs were simple in 28 (63.6%) and complex in 16 (36.4%). Heterogeneous R43Q mutation of gamma-aminobutyric acid A receptor g2 subunit occurred significantly more often in the patient group (36%) than in the control group (2%); p &#x003C; 0.001. The homozygous mutation carrier status was not different in the 2 groups. Family history of febrile convulsion and epilepsy was significantly higher in the study group than in controls (p &#x003C; 0.01). [<xref ref-type="bibr" rid="CIT0001">1</xref>]</p>
<p>COMMENTARY. The febrile seizure trait is inherited as a polygenic or multifactorial model or an autosomal dominant pattern with reduced penetrance [<xref ref-type="bibr" rid="CIT0002">2</xref>]. Mutations of several genes have been linked to febrile seizures, including voltage-gated sodium, calcium, and potassium, and ligand-gated ion channels, nicotinic cholinergic receptor and gamma-aminobutyric acid A (GABAa) receptor. R43Q mutation of the GABAa receptor g2-subunit is involved in the cause of absence epilepsy and febrile seizure [<xref ref-type="bibr" rid="CIT0003">3</xref>]. Twin studies reveal distinct genetic factors for different FS subtypes and sub-syndromes, especially FS+ [<xref ref-type="bibr" rid="CIT0004">4</xref>].</p>
</body>
<back>
<ref-list>
<ref id="CIT0001">
<label>1</label>
<element-citation publication-type="journal">
<person-group person-group-type="author">
<name>
<surname>Hancili</surname>
<given-names>S</given-names>
</name>
<name>
<surname>&#x00D6;nal</surname>
<given-names>ZE</given-names>
</name>
<name>
<surname>Ata</surname>
<given-names>P</given-names>
</name>
<name>
<surname>Karatoprak</surname>
<given-names>EY</given-names>
</name>
<name>
<surname>G&#x00FC;rb&#x00FC;z</surname>
<given-names>T</given-names>
</name>
<name>
<surname>Bostanci</surname>
<given-names>M</given-names>
</name>
<etal/>
</person-group>
<article-title>The GABAa receptor g2 subunit (R43Q) mutation in febrile seizures</article-title>
<source>Pediatr Neurol</source>
<year>2014</year>
<month>Apr</month>
<volume>50</volume>
<issue>4</issue>
<fpage>353</fpage>
<lpage>6</lpage>
<pub-id pub-id-type="doi">10.1016/j.pediatrneurol.2014.01.002</pub-id>
<pub-id pub-id-type="pmid">24630281</pub-id>
</element-citation>
</ref> 
<ref id="CIT0002">
<label>2</label>
<element-citation publication-type="journal">
<person-group person-group-type="author">
<name>
<surname>Nakayama</surname>
<given-names>J</given-names>
</name>
</person-group>
<article-title>Progress in searching for the febrile seizure susceptibility genes</article-title>
<source>Brain Dev</source>
<year>2009</year>
<month>May</month>
<volume>31</volume>
<issue>5</issue>
<fpage>359</fpage>
<lpage>65</lpage>
<pub-id pub-id-type="doi">10.1016/j.braindev.2008.11.014</pub-id>
<pub-id pub-id-type="pmid">19201561</pub-id>
</element-citation>
</ref> 
<ref id="CIT0003">
<label>3</label>
<element-citation publication-type="journal">
<person-group person-group-type="author">
<name>
<surname>Wallace</surname>
<given-names>RH</given-names>
</name>
<name>
<surname>Marini</surname>
<given-names>C</given-names>
</name>
<name>
<surname>Petrou</surname>
<given-names>S</given-names>
</name>
<name>
<surname>Harkin</surname>
<given-names>LA</given-names>
</name>
<name>
<surname>Bowser</surname>
<given-names>DN</given-names>
</name>
<name>
<surname>Panchal</surname>
<given-names>RG</given-names>
</name>
<etal/>
</person-group>
<article-title>Mutant GABA(A) receptor gamma2-subunit in childhood absence epilepsy and febrile seizures</article-title>
<source>Nat Genet</source>
<year>2001</year>
<month>May</month>
<volume>28</volume>
<issue>1</issue>
<fpage>49</fpage>
<lpage>52</lpage>
<pub-id pub-id-type="doi">10.1038/ng0501-49</pub-id>
<pub-id pub-id-type="pmid">11326275</pub-id>
</element-citation>
</ref> 
<ref id="CIT0004">
<label>4</label>
<element-citation publication-type="journal">
<person-group person-group-type="author">
<name>
<surname>Eckhaus</surname>
<given-names>J</given-names>
</name>
<name>
<surname>Lawrence</surname>
<given-names>KM</given-names>
</name>
<name>
<surname>Helbig</surname>
<given-names>I</given-names>
</name>
<name>
<surname>Bui</surname>
<given-names>M</given-names>
</name>
<name>
<surname>Vadlamudi</surname>
<given-names>L</given-names>
</name>
<name>
<surname>Hopper</surname>
<given-names>JL</given-names>
</name>
<etal/>
</person-group>
<article-title>Genetics of febrile seizure subtypes and syndromes: a twin study</article-title>
<source>Epilepsy Res</source>
<year>2013</year>
<month>Jul</month>
<volume>105</volume>
<issue>1-2</issue>
<fpage>103</fpage>
<lpage>9</lpage>
<pub-id pub-id-type="doi">10.1016/j.eplepsyres.2013.02.011</pub-id>
<pub-id pub-id-type="pmid">23522981</pub-id>
</element-citation>
</ref>
</ref-list>
</back>
</article>