PPIB
The protein encoded by this gene is a cyclosporine-binding protein and is mainly located within the endoplasmic reticulum. It is associated with the secretory pathway and released in biological fluids. This protein can bind to cells derived from T- and B-lymphocytes, and may regulate cyclosporine A-mediated immunosuppression. Variants have been identified in this protein that give rise to recessive forms of osteogenesis imperfecta.
Full Name
peptidylprolyl isomerase B
Alternative Names
Cyclophilin B, CYP S1, CYPB, PPIase B, Rotamase B, S cyclophilin, SCYLP
Function
PPIase that catalyzes the cis-trans isomerization of proline imidic peptide bonds in oligopeptides and may therefore assist protein folding.
Biological Process
Bone developmentManual Assertion Based On ExperimentIMP:UniProtKB
Chaperone-mediated protein foldingManual Assertion Based On ExperimentIDA:UniProtKB
Neutrophil chemotaxisManual Assertion Based On ExperimentIDA:UniProtKB
Positive regulation by host of viral genome replicationManual Assertion Based On ExperimentIMP:AgBase
Positive regulation by host of viral processManual Assertion Based On ExperimentIMP:AgBase
Positive regulation of multicellular organism growthManual Assertion Based On ExperimentIMP:UniProtKB
Protein foldingManual Assertion Based On ExperimentIBA:GO_Central
Protein peptidyl-prolyl isomerizationManual Assertion Based On ExperimentIDA:UniProtKB
Protein stabilizationManual Assertion Based On ExperimentIMP:UniProtKB
Cellular Location
Virion
(Microbial infection).
Endoplasmic reticulum lumen
Melanosome
Identified by mass spectrometry in melanosome fractions from stage I to stage IV (PubMed:17081065).
Involvement in disease
Osteogenesis imperfecta 9 (OI9):
A form of osteogenesis imperfecta, a connective tissue disorder characterized by low bone mass, bone fragility and susceptibility to fractures after minimal trauma. Disease severity ranges from very mild forms without fractures to intrauterine fractures and perinatal lethality. Extraskeletal manifestations, which affect a variable number of patients, are dentinogenesis imperfecta, hearing loss, and blue sclerae. OI9 is a severe autosomal recessive form of the disorder.
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