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Family with sequence similarity 171 member B (FAM171B) is a protein encoded by the FAM171B gene, characterized as a single-pass type I membrane and polyglutamine protein[10][2]. It is widely expressed in the brain and nervous system, showing cytoplasmic and vesicular localization, particularly in neurons, and may play a role in vesicle trafficking[2][10]. FAM171B is increasingly recognized as a biomarker for pulmonary arterial hypertension, mediating immune cell infiltration and response in the disease microenvironment, but its precise molecular function remains incompletely understood[4][8][5]. The gene has also been implicated in congenital disorders (gastroschisis, otopalatodigital syndrome) and may be involved in chemoresistance and neurodegenerative mechanisms based on protein family properties[2][6][5]. No drugs are currently known to target FAM171B directly, and its mechanism in therapeutic interventions is not yet established.
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