Women
tend to be protected from diseases
related to
genes on the X because female cells randomly inactivate one of the X chromosomes, and that leaves some cells with a normal copy up and running.
Rare genetic diseases like LFS are good study models because they
tend to proceed from a change in a single
gene, as opposed to many, overlapping changes seen in more
related common diseases, in this case more common, non-inherited bone cancers.