PDE6B gene
The PDE6B gene encodes a protein subunit essential for rod cell function, which allows for vision in low-light conditions.
Normal Function
The PDE6B gene provides instructions for making a protein that is one part (the beta subunit) of a protein complex called cGMP-PDE. This complex is found in specialized light receptor cells called rods. Rods transmit visual signals from the eye to the brain specifically in low-light conditions.
When light enters the eye, cGMP-PDE is activated, breaking down molecules called GMP within the rod cell. This triggers channels on the cell membrane to close, resulting in the transmission of signals to the brain that are interpreted as vision.
Health Conditions Related to Genetic Changes
Autosomal dominant congenital stationary night blindness
At least one mutation in the PDE6B gene has been found to cause autosomal dominant congenital stationary night blindness. This mutation (His258Asp or H258N) impairs the normal function of the cGMP-PDE complex, causing it to be constantly turned on (constitutively active). Because the complex is always active, the signals that rod cells send to the brain are constantly occurring, even in bright light. The brain perceives this information as not meaningful, resulting in night blindness.
Retinitis pigmentosa
MedlinePlus Genetics provides information about Retinitis pigmentosa.
Other Names for This Gene
- GMP-PDE beta
- PDE6B_HUMAN
- PDEB
- phosphodiesterase 6B, cGMP-specific, rod, beta
- rod cGMP-phosphodiesterase beta-subunit
- rod cGMP-specific 3',5'-cyclic phosphodiesterase subunit beta
Genomic Location
The PDE6B gene is found on chromosome 4.
