Rab escort protein

Rab escort protein (REP) is a type of protein that plays a crucial role in the cellular biology of organisms. It is involved in the transport of proteins within the cell, specifically in the process of prenylation, a modification that allows proteins to attach to cell membranes.

Function

Rab escort protein is named for its role in escorting Rab GTPases, a family of proteins involved in intracellular membrane trafficking. The REP binds to the Rab protein and presents it to the geranylgeranyl transferase enzyme for prenylation. This modification allows the Rab protein to attach to the cell membrane and carry out its function in protein transport.

Structure

The structure of Rab escort protein is characterized by a long, flexible polypeptide chain. This allows it to bind to both the Rab protein and the geranylgeranyl transferase enzyme simultaneously, facilitating the prenylation process.

Clinical significance

Mutations in the gene encoding Rab escort protein can lead to a variety of diseases. For example, defects in REP1, one of the two human Rab escort proteins, are associated with Choroideremia, a rare genetic disorder that causes progressive vision loss.

See also

• Rab protein
• Prenylation
• Choroideremia

References

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