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P4hb

Function

This multifunctional protein catalyzes the formation, breakage and rearrangement of disulfide bonds. At the cell surface, seems to act as a reductase that cleaves disulfide bonds of proteins attached to the cell. May therefore cause structural modifications of exofacial proteins. Inside the cell, seems to form/rearrange disulfide bonds of nascent proteins. At high concentrations and following phosphorylation by FAM20C, functions as a chaperone that inhibits aggregation of misfolded proteins. At low concentrations, facilitates aggregation (anti-chaperone activity). May be involved with other chaperones in the structural modification of the TG precursor in hormone biogenesis. Also acts as a structural subunit of various enzymes such as prolyl 4-hydroxylase and microsomal triacylglycerol transfer protein MTTP (By similarity). Receptor for LGALS9; the interaction retains P4HB at the cell surface of Th2 T helper cells, increasing disulfide reductase activity at the plasma membrane, altering the plasma membrane redox state and enhancing cell migration (PubMed:21670307).

Post-translational modifications

Phosphorylation of Ser-359 by FAM20C is induced by endoplasmic reticulum stress and results in a functional switch from oxidoreductase to molecular chaperone. It also promotes interaction with ERN1.

Sequence Similarities

Belongs to the protein disulfide isomerase family.

Tissue Specificity

In the mammary gland, expressed at higher levels in lactating mice than in virgin mice.

Cellular localization

Alternative names

Pdia1, P4hb, Protein disulfide-isomerase, PDI, Cellular thyroid hormone-binding protein, Endoplasmic reticulum resident protein 59, Prolyl 4-hydroxylase subunit beta, p55, ER protein 59, ERp59

swissprot:P09103 entrezGene:18453