Clinical and molecular characterization of a transmitted reciprocal translocation t(1;12)(p32.1;q21.3) in a family co-segregating with mental retardation, language delay, and microcephaly
2011

Study of a Genetic Translocation Linked to Mental Retardation

Sample size: 3 publication 10 minutes Evidence: moderate

Author Information

Author(s): Liao Hsiao-Mei, Fang Jye-Siung, Chen Yann-Jang, Wu Kuang-Lun, Lee Kuei-Fang, Chen Chia-Hsiang

Primary Institution: National Tsing-Hua University

Hypothesis

The translocation may enhance the expression of the ALX1 gene via the position effect and result in the clinical symptoms of this family.

Conclusion

The translocation may enhance the expression of the ALX1 gene, leading to the clinical symptoms observed in the family.

Supporting Evidence

  • The translocation was identified in a family with severe mental retardation, language delay, and microcephaly.
  • Breakpoints were mapped using advanced genetic techniques.
  • The study suggests that the translocation may enhance ALX1 gene expression.

Takeaway

This study found a genetic change in a family that might be causing problems with learning and development in the children.

Methodology

Fluorescence in situ hybridization (FISH), long-range PCR, and direct sequencing were used to map the breakpoints on chromosomes 1p and 12q.

Limitations

The maternal grandmother and the affected mother's younger brother were not available for investigation of the origin of the translocation.

Participant Demographics

A Taiwanese family with a mother and her two affected boys.

Statistical Information

P-Value

p<0.05

Digital Object Identifier (DOI)

10.1186/1471-2350-12-70

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