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While the underlying pathogenesis has not been elucidated in patients with isolated lesions, biallelic NF1 inactivation has been identified in melanocytes from CALMs in patients with NF1, and in McCuneAlbright syndrome, affected skin contains an activating mutation in the gene that encodes G s (see Chs 61 & 65)

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Same three-peptide core with one key caveat: the inclusion of KPV peptide

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It is abundant in human tissues, but subject to decline with aging

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