dominant negative

dominant negative
A mutation which is capable of exerting an effect even when only one copy is present, as in a heterozygote. Usually explained as a mutation that disrupts one subunit of a multimeric protein thus making the whole complex dysfunctional. Alternatively the mutated protein may compete with the normal protein produced by the other allele so that the overall activity is reduced below a critical threshold level and function is abnormal. The latter explanation only holds if the normal product of two genes is necessary for normal function (haplo-insufficiency) although experimental overexpression of an inactive form may reduce function by more than 50%.

Dictionary of molecular biology. 2004.

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