The Lyon hypothesis is a concept in genetics that proposes that in females, one copy of the X chromosomes is randomly inactivated during embryonic development. The spelling of "Lyon hypothesis" is pronounced as /liːɒn haɪˈpɒθəsɪs/. In this transcription, the stress is on the first syllable of "Lyon," followed by the stress on the "hypo" of "hypothesis." The "y" in "Lyon" is pronounced as a long "i" sound (like "eye"), while the "o" is pronounced with a short "oh" sound. The final "s" in "hypothesis" is pronounced as a "z" sound.
The Lyon hypothesis, also known as the Lyonization or Lyon's Law, is a concept in genetics that explains the random inactivation of one of the two X chromosomes in female mammalian cells. Developed by Mary Lyon, the hypothesis states that during early embryonic development, one of the two X chromosomes present in female cells becomes inactivated, leading to a single active X chromosome. This process occurs in order to maintain gene dosage equality between males and females, as males only have one X chromosome.
The Lyon hypothesis proposes that X-chromosome inactivation is a stochastic event influenced by genetic and epigenetic factors. The choice of which X chromosome to inactivate is believed to be random and occurs independently in each cell in the early stages of development. Consequently, the inactivation is clonally propagated to all progeny cells derived from that particular cell line.
This phenomenon of X-chromosome inactivation has crucial implications in the understanding of X-linked genetic disorders, as it explains why females with two X chromosomes can still experience diseases caused by mutations on the X chromosome. It also sheds light on why some diseases may exhibit skewed X-inactivation patterns, leading to varied clinical presentations among affected individuals.
The Lyon hypothesis has revolutionized the field of genetics, providing a theoretical framework to understand the chromosomal differences between males and females and their implications for genetic expression in various developmental and disease contexts.