Identification of a novel FBN1 gene mutation in a Chinese family with Marfan syndrome
2011

New FBN1 Gene Mutation Found in Marfan Syndrome Family

Sample size: 6 publication Evidence: moderate

Author Information

Author(s): Meng Bo, Li Hongyi, Yang Tao, Huang Shangzhi, Sun Xian, Yuan Huiping

Primary Institution: 2nd Affiliated Hospital of Harbin Medical University

Hypothesis

To identify the mutation in the fibrillin-1 gene (FBN1) in a Chinese family with Marfan syndrome (MFS).

Conclusion

A novel p.S1235P mutation in FBN1 was identified as the causative mutation for Marfan syndrome in this family.

Supporting Evidence

  • A novel heterozygous c.3703T>C change in exon 29 of FBN1 was detected.
  • The mutation was present in affected family members but absent in healthy controls.
  • The mutant residue is highly conserved among mammalian species.

Takeaway

Scientists found a new change in a gene that causes Marfan syndrome in a family, which helps understand the disease better.

Methodology

Genomic DNA was extracted from family members and healthy controls, and the FBN1 gene was sequenced to identify mutations.

Limitations

The study had a small sample size and was limited to one family.

Participant Demographics

The study involved a four-generation Chinese family with Marfan syndrome.

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