With emerging evidence that endogenous macromolecule damage induced by formaldehyde and aldehyde derivatives may be the causative force underlying FA [357,358], a central challenge will be to determine how molecular defects in FANCJ and the 20+ other proteins implicated in the FA pathway are responsible for the characteristic disease outcomes, i.e., accelerated decline of the hematopoietic stem cell compartment and other features of aging [359]. Here, BRIP1 is linked to Friedreich ataxia.