HSPD1(heat shock protein family D (Hsp60) member 1) encodes a member of the chaperonin family. The encoded mitochondrial protein may function as a signaling molecule in the innate immune system. This protein is essential for the folding and assembly of newly imported proteins in the mitochondria. HSPD1 is adjacent to a related family member and the region between the 2 genes functions as a bidirectional promoter. Several pseudogenes have been associated with HSPD1. Two transcript variants encoding the same protein have been identified for HSPD1. Mutations associated with this gene cause autosomal recessive spastic paraplegia 13.基因ID为:3329;蛋白质ID:P10809