A novel mutation in STK11 gene is associated with Peutz-Jeghers Syndrome in Indian patients
2006

Mutation in STK11 Gene Linked to Peutz-Jeghers Syndrome in Indian Patients

Sample size: 16 publication Evidence: moderate

Author Information

Author(s): Thakur Nikita, Reddy D Nageshwar, Rao G Venkat, Mohankrishna P, Singh Lalji, Chandak Giriraj R

Primary Institution: Centre for Cellular and Molecular Biology

Hypothesis

Is there a novel mutation in the STK11 gene associated with Peutz-Jeghers Syndrome in Indian patients?

Conclusion

This study is the first to report a mutation in the STK11 gene in Indian Peutz-Jeghers Syndrome patients, suggesting that known mutations do not account for the disease in many cases.

Supporting Evidence

  • A novel pathogenic mutation (c.790_793 delTTTG) was identified in one index patient and three family members.
  • The mutation leads to a frame-shift and premature termination of the STK11 protein.
  • The study suggests that large genomic deletions or another locus may be associated with Peutz-Jeghers Syndrome in India.

Takeaway

Scientists found a new change in a gene that might cause a rare disease in some Indian families, but many cases still don't have a clear cause.

Methodology

Sequencing of the promoter and coding regions of the STK11 gene in affected members from ten Indian Peutz-Jeghers Syndrome families.

Limitations

The study does not account for all cases of Peutz-Jeghers Syndrome in India, and the presence of other potential genetic factors remains unexplored.

Participant Demographics

Affected members from ten well-characterized Indian Peutz-Jeghers Syndrome families.

Digital Object Identifier (DOI)

10.1186/1471-2350-7-73

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