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Bellanti et al., 2022
Moreover, it has an in vitro pharmacological profile that mimics the signaling of native GIP at the GIPR, but it is biased at the GLP-1R to favor cyclic AMP (cAMP) generation over -arrestin recruitment 16
Together they produce a larger, synergistic GH pulse than either alone
Treatment consists of replacement of kidney tubular losses, symptomatic management of systemic complications, and specific therapy directed at the basic defect, i.e., lysosomal cystine accumulation
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