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Rab23 is a novel member of large Rab small GTPase family. It plays important roles during embryonic development. The brain-enriched Rab23 is the only Rab protein that is known to have a distinct function during central nervous system development, playing an essential role as a negative regulator of the Sonic Hedgehog (Shh) pathway. RAB23 has recently been identified in mesangial cells (MCs), and could serve as a biomarker that indicates the severity of FSGS ( focal segmental glomerulosclerosis). Mutations in RAB23 may result in ACPS2 ( acrocephalopolysyndactyly type 2).