Werner Syndrome Protein - Unwinding Function to Explain Disease
Abstract
Werner syndrome (WS) is one of three heritable human genetic instability/cancer predisposition syndromes that result from mutations in a member of the gene family encoding human RecQ helicases. Cellular defects are a prominent part of the WS phenotype. Here we review recent work to identify in vi-vo functions of the WS protein and discuss how loss of function leads to cellular defects. These new results provide clues to the origin of cell lineage-specific defects in WS patients and suggest a broader role for Werner protein function in determining disease risk in the general population.
Domains
Cancer
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