The PSMB gene is responsible for creating a harlequin pattern in the coats of Great Danes, which displays as a white coat with darker pigmented patches. Harlequin can only be expressed if the Merle gene is present, and the PSMB gene is said to be a modifier of the Merle gene. The coat pattern is due to an amino acid substitution in PSMB7, which causes the proteins function to be impaired. This impaired protein may be unable to degrade PMEL proteins that have been improperly spliced, which affects eumelanin synthesis and causes the harlequin phenotype. Some harlequin dogs may have brindle, sable, or gray patches (dependent on genotype and other coat color genes).
PSMB7
Dominant
https://pubmed.ncbi.nlm.nih.gov/21256207/ https://doi.org/10.1111/age.13154 http://www.doggenetics.co.uk/harlequin.html
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