Novel mutations of CHST6 in Iranian patients with macular corneal dystrophy
2008

Mutations in CHST6 Gene Linked to Macular Corneal Dystrophy in Iran

Sample size: 20 publication Evidence: moderate

Author Information

Author(s): Birgani Shiva Akbari, Salehi Zivar, Houshmand Masoud, Mohamadi Mohamad Javad, Promehr Leila Azizade, Mozafarzadeh Zahra

Primary Institution: Guilan University of Medical Science

Hypothesis

To characterize mutations within the carbohydrate sulfotransferase 6 (CHST6) gene in Iranian subjects with macular corneal dystrophy.

Conclusion

CHST6 mutations are responsible for the pathogenesis of macular corneal dystrophy in Iranian patients.

Supporting Evidence

  • The study identified 11 different mutations in the CHST6 gene.
  • Six of the mutations were novel missense mutations.
  • One mutation was a novel nonsense mutation.
  • The majority of mutations were located in the binding sites of the protein.

Takeaway

The study found new changes in a gene that causes a type of eye disease in some people from Iran.

Methodology

Genomic DNA was extracted from blood samples of 20 affected patients and 60 healthy volunteers, followed by PCR and sequencing of the CHST6 coding region.

Limitations

Some cases of macular corneal dystrophy could not be explained by mutations in the coding region of CHST6.

Participant Demographics

20 patients from 12 unrelated Iranian families, all older than 25 years.

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