MC4R and obesity due to melanocortin 4 receptor deficiency: Since setmelanotide, a melanocortin-4-receptor (MC4R) agonist, is being evaluated as anti-obesity treatment for certain cases with monogenic obesity [30], including cases with an SH2B1 variant, our cases with distal 16p11.2 BP2-BP3 deletions are currently eligible for these clinical trials.