The data summarized here support the concept that certain variantly spliced BRCA2 transcripts from the 5′ segment of BRCA2 may confer BRCA2 activity for survival in the presence of PVs in the region spanning exons 3 to 7 or beyond, but do not prevent clinical manifestations of FA or malignant transformation. The gene discussed is BRCA2; the disease is Friedreich ataxia.