Home LiteratureArticle Details
PMID: 22633631 Published · ppublish English

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

Pediatric neurology ·Vol. 46 ·No. 6 ·2013-03-29

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

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.

Article Info
Journal
Pediatric neurology
Abbr.
Pediatr Neurol
Published
2013-03-29
Indexed
2012-05-28
Updated
2012-05-28
Language
English
Country/Region
United States
NLM ID
8508183
Analysis Services
Analysis Services

Contact

No. 2 Wenbo Road, Zhangqiu District, Jinan, Shandong

Qilu Normal University · Genelibs Bioinformatics Lab

750 Shunhua Rd, Jinan

2F, Bldg F, University Science Park

Tel: 0531-88819269

WeChat Official Account

Follow our WeChat subscription account for real-time updates and the latest in medical and biological research.


Business Email

E-mail: product@genelibs.com