GrpE protein homolog 2, mitochondrial (GRPEL2)

GrpE protein homolog 2, mitochondrial (GRPEL2) is a component of the mitochondrial import machinery and functions as a nucleotide exchange factor (NEF) for mitochondrial Hsp70 (mtHsp70)[1][4]. It facilitates the exchange of ADP for ATP on mtHsp70, thereby regulating its chaperone activity during mitochondrial protein import and folding. GRPEL2 can form homo- and hetero-oligomeric complexes with its paralog GRPEL1, together helping to sustain mitochondrial function under stress conditions. While GRPEL1 is essential for basic mitochondrial function, GRPEL2 appears to be more important under oxidative stress, conferring stress resilience to mitochondrial protein quality control. GRPEL2 is not a traditional therapeutic target and currently has no known drugs interacting with it, but its deficiency has been associated with disorders such as Perrault Syndrome and impaired mitochondrial homeostasis in experimental models[1][4].

Other names

GrpE like 2, mitochondrialGRPEL2GRPE2_HUMANDKFZp451C205Mt-GrpE#2FLJ23713grpE protein homolog 2, mitochondrial