A novel DSPP mutation is associated with type II dentinogenesis Imperfecta in a chinese family
2007

New Mutation Linked to Tooth Defect in Chinese Family

Sample size: 18 publication Evidence: high

Author Information

Author(s): Zhang Xianqin, Chen Lanying, Liu Jingyu, Zhao Zhen, Qu Erjun, Wang Xiaotao, Chang Wei, Xu Chengqi, Wang Qing K, Liu Mugen

Primary Institution: Huazhong University of Science and Technology

Hypothesis

Is there a novel mutation in the DSPP gene associated with type II dentinogenesis imperfecta in a Chinese family?

Conclusion

The mutation p.Pro17Ser in the DSPP gene is responsible for type II dentinogenesis imperfecta in the studied Chinese family.

Supporting Evidence

  • The mutation was found in all affected individuals but not in normal family members or 100 controls.
  • The Pro17 residue is highly conserved across different species.
  • Linkage analysis confirmed that the disease-causing gene is linked to the DSPP gene.

Takeaway

Scientists found a new change in a gene that causes a tooth problem in a family from China. This change makes their teeth look different and not work well.

Methodology

Linkage analysis and direct DNA sequencing were used to identify the mutation in the DSPP gene.

Participant Demographics

The study involved a four-generation Chinese family with 18 living members, including 8 affected individuals.

Digital Object Identifier (DOI)

10.1186/1471-2350-8-52

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