The vast majority of the variants found in patients are inactivating, with more than 400 unique variants reported [3], and they are associated with pseudohypoparathyroidism type 1A (PHP1A), pseudopseudohypoparathyroidism (PPHP), or progressive osseous heteroplasia (POH) (for review [4]), which is currently renamed as inactivating PTH/PTHrP signaling disorder type 2 (iPPSD2) [2]. This evidence concerns the gene PTHLH and pseudohypoparathyroidism type 1A.