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In addition, there is increasing evidence that genetic modifiers besides CFTR modulate the frequency and severity of the disease.
One example is mannan-binding lectin, which is involved in innate immunity by facilitating phagocytosis of microorganisms.
Polymorphisms in one or both mannan-binding lectin alleles that result in lower circulating levels of the protein are associated with a threefold higher risk of end-stage lung disease, as well as an increased burden of chronic bacterial infections.

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