Osteogenesis imperfecta is a phenotypically and molecularly heterogeneous group of
inherited connective tissue disorders that share similar skeletal abnormalities causing bone …

The endoplasmic reticulum (ER) is the major folding compartment for secreted and
membrane proteins and is the site of a specific chaperone system, the calnexin cycle, for …

Cyclophilin A (CyPA) is a ubiquitously distributed protein belonging to the immunophilin
family. CyPA has peptidyl prolyl cis-trans isomerase (PPIase) activity, which regulates …

The extracellular matrix represents a complex alloy of variable members of diverse protein
families defining structural integrity and various physiological functions. The most abundant …

A new paradigm has emerged for osteogenesis imperfecta as a collagen-related disorder.
The more prevalent autosomal dominant forms of osteogenesis imperfecta are caused by …

FK506 and rapamycin are related immunosuppressive compounds that block helper T cell
activation by interfering with signal transduction. In vitro, both drugs bind and inhibit the …

Complex processes are involved in the protection of cells and organisms by heat shock
proteins (hsps). Aberrant protein production due to high temperatures is prevented by the …

Posttranslational modifications can cause profound changes in protein function. Typically,
these modifications are reversible, and thus provide a biochemical on-off switch. In contrast …

Osteogenesis imperfecta is a genetic disorder characterized by low bone mass, decreased
bone strength, increased bone fragility, and shortened stature. Autosomal dominant …

Tendons and ligaments are connective tissues that guide motion, share loads, and transmit
forces in a manner that is unique to each as well as the anatomical site and biomechanical …