FKBP prolyl isomerase 10

Normal function

Den FKBP10 gene provides instructions for making a protein that is found in a cell structure called the endoplasmic reticulum, which is involved in protein production, processing, and transport. The FKBP10 protein (formerly known as FKBP65) is important for the correct processing of complex molecules called collagen and elastin, which are part of the intricate lattice of proteins and other molecules that forms in the spaces between cells (the extracellular matrix). This matrix provides structure and strength to connective tissues that support the body's joints and organs.

In the extracellular matrix, collagen molecules are cross-linked to one another to form long, thin fibrils. The formation of cross-links results in very strong collagen fibrils. The FKBP10 protein attaches to collagen molecules and plays a role in their cross-linking. It is thought to be involved in a reaction called hydroxylation that modifies a particular region of the collagen molecule and is necessary for cross-linking of the molecules.

The FKBP10 protein is also involved in the formation of elastin. In particular, FKBP10 helps with the proper folding of a protein called tropoelastin. Multiple copies of tropoelastin attach to one another to make elastin. Elastin is the major component of elastic fibers, which provide strength and elasticity to connective tissues as part of the extracellular matrix.

Conditions referring to this gene or chromosome

• Osteogenesis imperfecta

• Kuskokwim syndrome

Other names

65 kDa FK506-binding protein

65 kDa FKBP

FK506 binding protein 10

FK506 binding protein 10, 65 kDa

FK506-binding protein 10

FKB10_HUMAN

FKBP-10

FKBP-65

FKBP6

FKBP65

FLJ20683

FLJ22041

FLJ23833

hFKBP65

immunophilin FKBP65

OI11

OI6

peptidyl-prolyl cis-trans isomerase FKBP10

peptidyl-prolyl cis-trans isomerase FKBP10 precursor

PPIASE

PPIase FKBP10

rotamase