GRPEL1 Human

The GRPEL1 gene encodes a protein consisting of 217 amino acids. This protein shares significant homology with its counterparts in other species, including an 88% identity with the rat GrpE homolog and 28% identity with the bacterial GrpE protein . The protein contains conserved residues that interact with HSP70 proteins, suggesting its role in facilitating nucleotide exchange .

GRPEL1 is an essential component of the Presequence translocase-associated motor (PAM) complex, which is required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner . It controls the nucleotide-dependent binding of mitochondrial HSP70 to substrate proteins .

The protein is involved in several key cellular processes:

• Protein Import: GRPEL1 interacts with mitochondrial HSP70, a molecular chaperone that assists in protein folding, translocation across membranes, and degradation .
• Nucleotide Exchange: It facilitates the exchange of nucleotides in HSP70, which is crucial for the proper functioning of the chaperone system .

GRPEL1 is ubiquitously expressed in various tissues, with particularly high levels in the heart, brain, liver, and kidney . It is localized in the mitochondrial matrix and nucleoplasm, indicating its role in mitochondrial function and possibly other cellular processes .

Mutations or dysregulation of GRPEL1 can potentially impact mitochondrial function, leading to various mitochondrial disorders. Understanding the role and mechanism of GRPEL1 can provide insights into the development of therapeutic strategies for these conditions.

Recombinant human GRPEL1 is used in various research applications to study its function and interactions. It is available from several biotechnology companies for use in laboratory experiments .