Brief Article
Copyright ©2010 Baishideng. All rights reserved
World J Gastroenterol. May 14, 2010; 16(18): 2278-2282
Published online May 14, 2010. doi: 10.3748/wjg.v16.i18.2278
Most common SLC25A13 mutation in 400 Chinese infants with intrahepatic cholestasis
Hai-Yan Fu, Shao-Ren Zhang, Hui Yu, Xiao-Hong Wang, Qi-Rong Zhu, Jian-She Wang
Hai-Yan Fu, Shao-Ren Zhang, Hui Yu, Xiao-Hong Wang, Qi-Rong Zhu, Jian-She Wang, Center for Pediatric Liver Diseases, Children’s Hospital of Fudan University, Shanghai 201102, China; Department of Pediatrics, Shanghai Medical College of Fudan University, 399 Wanyuan Road, Minhang District, Shanghai 201102, China
Author contributions: Fu HY and Zhang SR collected the samples and performed the gene tests; Fu HY analyzed the data and wrote the first graft of this paper; Wang JS designed the research, revised the paper and approved the final paper to be published; All the authors contributed to the research design, data collection and analysis.
Supported by National Natural Science Foundation of China, No. 30672257 and No. 30973230; and Shanghai Public Health Key Subject Construction, No. 08GWZX0102
Correspondence to: Jian-She Wang, Professor, Center for Pediatric Liver Diseases, Children’s Hospital of Fudan University, 399 Wanyuan Road, Minhang District, Shanghai 201102, China. jshwang@shmu.edu.cn
Telephone: +86-21-64931171 Fax: +86-21-64931171
Received: January 16, 2010
Revised: February 23, 2010
Accepted: March 1, 2010
Published online: May 14, 2010
Abstract

AIM: To establish the real time fluorescence polymerase chain reaction (RT-PCR) with dual labeled probes for fast detection of SLC25A13 gene mutation 851del4.

METHODS: Four hundred infants (< 1 year of age) with unexplained intrahepatic cholestasis from 18 provinces or municipalities in China were enrolled in this study for detecting their SLC25A13 gene mutation 851del4. Suitable primers and fluorescence-labeled probes for detecting SLC25A13 gene mutation 841del4 were designed. Normal and mutant sequences were detected by PCR with two fluorescence-labeled probes. After a single RT-PCR, results were obtained by analyzing the take-off curves. Twenty-four positive and 14 negative samples were retested by direct sequencing.

RESULTS: Eight homozygous and 30 heterozygous mutations were detected in 46 mutant alleles with a 851del4 mutation rate of 5.8% (46/800). Twenty-six and 20 mutant alleles were observed respectively, in 474 and 242 alleles from the intermediate and southern areas of China. No mutant allele was detected in 84 alleles from northern China. Twenty-four positive samples including 4 homozygous and 20 heterozygous mutations, and 14 negative samples were retested by direct sequencing, which confirmed that the accuracy of RT-PCR was 100%.

CONCLUSION: RT-PCR can detect the mutation 851del4 in infants with intrahepatic cholestasis with an accuracy of 100%.

Keywords: 851del4 mutation; Neonatal intrahepatic cholestasis; Real-time fluorescent polymerase chain reaction; SLC25A13 gene