FKBP prolyl isomerase 10
अंतिम अद्यतन 11 Apr 2023
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यह 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.
Normal function
यह 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
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
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अधिक पठन और संदर्भ
- FKBP prolyl isomerase 10; MedlinePlus, National Library of Medicine.
- Barnes AM, Cabral WA, Weis M, Makareeva E, Mertz EL, Leikin S, Eyre D, Trujillo C, Marini JC. Absence of FKBP10 in recessive type XI osteogenesis imperfecta leads to diminished collagen cross-linking and reduced collagen deposition in extracellular matrix. Hum Mutat. 2012 Nov;33(11):1589-98. doi: 10.1002/humu.22139. Epub 2012 Jul 16.
- Ishikawa Y, Vranka J, Wirz J, Nagata K, Bachinger HP. The rough endoplasmic reticulum-resident FK506-binding protein FKBP65 is a molecular chaperone that interacts with collagens. J Biol Chem. 2008 Nov 14;283(46):31584-90. doi: 10.1074/jbc.M802535200. Epub 2008 Sep 10.
- Miao M, Reichheld SE, Muiznieks LD, Huang Y, Keeley FW. Elastin binding protein and FKBP65 modulate in vitro self-assembly of human tropoelastin. Biochemistry. 2013 Nov 5;52(44):7731-41. doi: 10.1021/bi400760f. Epub 2013 Oct 24.
- Schwarze U, Cundy T, Pyott SM, Christiansen HE, Hegde MR, Bank RA, Pals G, Ankala A, Conneely K, Seaver L, Yandow SM, Raney E, Babovic-Vuksanovic D, Stoler J, Ben-Neriah Z, Segel R, Lieberman S, Siderius L, Al-Aqeel A, Hannibal M, Hudgins L, McPherson E, Clemens M, Sussman MD, Steiner RD, Mahan J, Smith R, Anyane-Yeboa K, Wynn J, Chong K, Uster T, Aftimos S, Sutton VR, Davis EC, Kim LS, Weis MA, Eyre D, Byers PH. Mutations in FKBP10, which result in Bruck syndrome and recessive forms of osteogenesis imperfecta, inhibit the hydroxylation of telopeptide lysines in bone collagen. Hum Mol Genet. 2013 Jan 1;22(1):1-17. doi: 10.1093/hmg/dds371. Epub 2012 Sep 4.
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