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Related Experiment Videos

Molecular basis for complement component 6 (C6) deficiency in rats and mice.

Deepak Bhole1, Gregory L Stahl

  • 1Center for Experimental Therapeutics and Reperfusion Injury, Department of Anesthesiology, Perioperative and Pain Medicine, Brigham & Women's Hospital, Harvard Medical School, 75 Francis Street, Boston, MA 02115, USA.

Immunobiology
|December 1, 2004
PubMed
Summary

Researchers identified the genetic causes of complement component C6 deficiency in rats and mice. C6 deficiency was linked to a deletion in rats and mutations in mice, unexpectedly revealing a coagulation defect in deficient animals.

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Area of Science:

  • Immunology
  • Genetics
  • Hematology

Background:

  • Complement component C6 (C6) is crucial for the lytic membrane attack complex in complement activation.
  • Rodent models deficient in C6 are used to study complement's role in human disease, but the genetic basis of these deficiencies is unknown.
  • Understanding C6 deficiency is vital for accurate animal modeling in immunological and disease research.

Purpose of the Study:

  • To elucidate the molecular basis of C6 deficiency in rat and mouse strains.
  • To develop genetic tests for identifying C6-deficient rodents.
  • To investigate potential secondary effects of C6 deficiency, such as coagulation abnormalities.

Main Methods:

  • Isolation and sequencing of rat C6 mRNA using EST-derived oligonucleotides.

Related Experiment Videos

  • Identification of gene mutations (deletion in rats, point mutations in mice) responsible for C6 deficiency.
  • Development of a PCR-based genotyping assay for rat C6 deficiency.
  • Assessment of tail bleeding times and platelet aggregation in C6-deficient and wild-type rodents.

    • Main Results:

    • A 31 bp deletion in exon 10 of the C6 gene was identified as the cause of deficiency in PVG rats (PVG/c-).
    • Four point mutations in the mouse C6 gene were found, potentially explaining C6 deficiency in the Peru-Coppock strain.
    • C6-deficient mice and rats exhibited prolonged bleeding times and reduced platelet aggregation, indicating a coagulation defect.
    • Treatment with purified rat C6 protein reversed the prolonged bleeding times in deficient animals.

    Conclusions:

    • The genetic underpinnings of C6 deficiency in specific rat and mouse strains have been identified.
    • A reliable PCR-based genotyping method for rat C6 deficiency has been established.
    • Complement component C6 deficiency is unexpectedly associated with a coagulation defect, the mechanism of which requires further investigation.