FKBP10 : FKBP prolyl isomerase 10
Description
The FKBP10 (FKBP prolyl isomerase 10) is a protein-coding gene located on chromosome 17.
The FKBP10 gene provides instructions for making a protein that is found in the endoplasmic reticulum, a cell structure involved in protein production, processing, and transport. This protein, known as FKBP10 or FKBP65, is crucial for the correct processing of collagen and elastin, complex molecules that form the extracellular matrix, providing structure and strength to connective tissues. FKBP10 attaches to collagen molecules and facilitates their cross-linking by participating in a hydroxylation reaction, modifying a specific region of the collagen molecule. Furthermore, FKBP10 aids in the proper folding of tropoelastin, a protein that assembles into elastin, the primary component of elastic fibers, which contribute to the elasticity and strength of connective tissues.
FKBP10 protein acts as a peptidyl-prolyl cis-trans isomerase (PPIase), which helps accelerate the folding of proteins during protein synthesis.
FKBP10 is also known as BRKS, BRKS1, FKBP65, OI11, OI6, PPIASE, TLH1, hFKBP65.
Associated Diseases
- Osteogenesis imperfecta, type XI
- Kuskokwim syndrome
- Bruck syndrome
- Bruck syndrome 1
- Osteogenesis imperfecta
