The key is that mutations occur but repeated mutations don't generally occur unless there's something very weird going on like in the case of Huntington's where there's a whole family of bad alleles and there's a biochemical quick which makes the mutations much more likely.
Pardon me if I'm being obtuse, but wouldn't we expect "a whole family of bad alleles" to be the usual case, since you can break a protein in any number of different ways?
I've heard that some fairly high percentage of hemophilia A and B cases are de novo mutations (a quick Google turned up this). I'm sure it's because hemophilia is pretty lethal and often doesn't get the chance to be inherited, but it's another case where mutation rates do seem to matter.
Yes, hemophilia is an example like Huntington's where there's a family of alleles. And of course, in that case, the allele is extremely lethal, killing a large fraction of males, and killing any female that is homozygous. So the allele has to stay really rare.
In general though for most proteins it is surprisingly difficult to break them. Most mutations will actually be neutral. They will be neutral either because the mutated codon actually codes for the same allele, or codes for a chemically similar allele, or because it is a section of the protein which ...
A recent entry from the West Hunters blog (written by Gregory Cochran and Henry Harpending with whom most LWers are probably already familiar with) caught my eye:
Seems quite coherent. It meshes well with findings that the more children parents have the less they subscribe to nurture, since they finally, possibly for the first time ever, get some hands on experience with the nurture (nurture as in stuff like upbringing not nurture as in lead paint) versus. nature issue. Note that today urban, educated, highly intelligent people are less likley to have children than possibly ever, how is this likley to effect intellectual fashions?
Perhaps somewhat related to this is also the transition in the past 150 years (the time frame depending on where exactly you live) from agricultural communities, that often raised livestock to urban living. What exactly "variation" and "heredity" might mean in a intuitive way thus comes another source short with no clear replacement.