A novel HSF4 gene mutation (p.R405X) causing autosomal recessive congenital cataracts in a large consanguineous family from Pakistan
2008

Mutation in HSF4 Gene Causes Cataracts in Pakistani Family

Sample size: 14 publication 10 minutes Evidence: moderate

Author Information

Author(s): Sajjad Naheed, Goebel Ingrid, Kakar Naseebullah, Cheema Abdul Majeed, Kubisch Christian, Ahmad Jamil

Primary Institution: Faculty of Biotechnology and Informatics, BUITEMS, Quetta, Pakistan

Hypothesis

The study investigates the genetic basis of autosomal recessive congenital cataracts in a consanguineous family from Pakistan.

Conclusion

The study identified a novel nonsense mutation in the HSF4 gene that causes autosomal recessive cataracts in a large consanguineous family from Pakistan.

Supporting Evidence

  • The disease phenotype co-segregated with markers flanking the HSF4 gene.
  • A maximum two-point LOD score of 5.6 was obtained for marker D16S421.
  • The identified mutation was not present in unaffected family members or in control samples.

Takeaway

A family from Pakistan has a special eye problem called cataracts because of a change in a gene called HSF4. This change stops the gene from working properly.

Methodology

Genetic linkage analysis and sequencing of the HSF4 gene were performed on family members.

Limitations

The study is limited to a single family, which may not represent the broader population.

Participant Demographics

The study involved a large consanguineous family from Pakistan with eight affected individuals.

Statistical Information

P-Value

p<0.05

Statistical Significance

p<0.05

Digital Object Identifier (DOI)

10.1186/1471-2350-9-99

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