[{"from": "human", "value": "Provide a brief overview of this molecule. [C][C][N][Branch2][Branch1][Ring1][C][C][C][Ring1][=Branch1][C][C][=Branch1][C][=O][N][C][C][C][Branch1][Branch1][C][C][Ring1][=Branch1][C@@H1][C][=C][Branch1][S][C][C][C][=C][Ring1][#Branch1][N][=C][C][=Branch1][Ring2][=C][Ring1][=Branch1][Br][C][=C][Branch1][=Branch1][C][=C][Ring1][#C][Br][Cl][C][=Branch1][C][=O][N]"}, {"from": "gpt", "value": "The molecule is a rare autosomal dominant disorder estimated to affect approximately one in 20 million individuals resulting in adverse symptoms associated with premature ageing: skeletal dysplasia, joint contractures, atherosclerosis, myocardial fibrosis/dysfunction, scleroderma-like cutaneous effects, lipoatrophy, alopecia, and a severe failure to thrive; HGPS is uniformly fatal. Mechanistically, HGPS is underpinned by a single heterozygous C-to-T mutation at position 1824 of the LMNA gene, which results in the accumulation of an aberrant farnesylated form of lamin A called progerin in the inner nuclear membrane. Lonafarnib is a farnesyl transferase (FTase) inhibitor (FTI), which reduces the farnesylation of numerous cellular proteins, including progerin; as progerin farnesylation is important for localization to the nuclear membrane, lonafarnib inhibits progerin accumulation and improves symptoms in HGPS patients.  Merck originally developed Lonafarnib and subsequently licensed it to Eiger Biopharmaceuticals Inc., which currently markets it under the trademark ZOKINVY\u2122. Lonafarnib was granted FDA approval on November 20, 2020, and is the first FDA-approved treatment for HGPS and other related progeroid laminopathies."}]