The capacity to blunder slightly is the real marvel of DNA. Without this special attribute, we would still be anaerobic bacteria and there would be no music.

Lewis Thomas

The sources of mutations

DNA replication and unrepaired DNA damage

Mutations arise via two major mechanisms: base misincorporation during replication of non-damaged DNA; and accumulation of DNA damage that has not been properly repaired, leading to mutation.

Deficiencies of co-replicative repair

The rate of nucleotide misincorporation during replication is monitored by the exonuclease activity of major DNA polymerases (Pol \(\delta\) for lagging strand, Pol \(\epsilon\) for leading-strand) and by co-replicative MMR (mismatch repair).

MMR

  • more proficient in removing replication errors on the lagging strand than on the leading strand
  • activity correlates with the chromatin structure

Deficiencies of other DNA repair mechanisms

Male and female germlines undergo similar numbers of mitotic cell divisions by the onset of puberty (~30 to 35 divisions). Thereafter the ratio of male to female cell divisions increases rapidly with age, because of frequent mitotic divisions of spermatogonial stem cells (an estimated 23 divisions per year) and the absence of mitosis of female germ cells over the same period.

Paternal mutations: 75-80%, no significant effect of paternal age

Variation in mutation rate

Estimation of germline mutation rate

Conrad

Variation along the genome

CpG to TpG

Deamination of a mC creates a T:G mismatch. Erroneous repair of these by BER to T:A will lead to mutation (independent of replication, track time rather than the number of cell divisions). Mismatches formed immediately before cell division and repaired until replication also lead to mutation (track cell divisions because BER is efficient at short timescales). During replication, the deamination rate is probably increased for single-stranded DNA, and the lagging strand is exposed to the single-strand conformation during replication stress. Moreover, mC may be an inferior template for polymerases and could lead to misincorporation opposite mC.

Variation across species

Models of the mutation process

Infinite-alleles model

Infinite-sites model

Finite-alleles model

Stepwise mutation model