Functions and pathologies of BiP and its interaction partners.
The endoplasmic reticulum (ER) is involved in a variety of essential and interconnected processes in human cells, including protein biogenesis, signal transduction, and calcium homeostasis. The central player in all these processes is the ER-lumenal polypeptide chain binding protein BiP that acts as a molecular chaperone. BiP belongs to the heat shock protein 70 (Hsp70) family and crucially depends on a number of interaction partners, including co-chaperones, nucleotide exchange factors, and signaling molecules. In the course of the last five years, several diseases have been linked to BiP and its interaction partners, such as a group of infectious diseases that are caused by Shigella toxin producing E. coli. Furthermore, the inherited diseases Marinesco-Sjögren syndrome, autosomal dominant polycystic liver disease, Wolcott-Rallison syndrome, and several cancer types can be considered BiP-related diseases. This review summarizes the physiological and pathophysiological characteristics of BiP and its interaction partners.
Dudek
J
J
Medical Biochemistry and Molecular Biology, Saarland University, 66421, Homburg, Germany.
Benedix
J
J
Cappel
S
S
Greiner
M
M
Jalal
C
C
Müller
L
L
Zimmermann
R
R
D002118:Calcium / Q000378:metabolism; D004721:Endoplasmic Reticulum / Q000378:metabolism; D000091342:Endoplasmic Reticulum Chaperone BiP; D004927:Escherichia coli Infections / Q000378:metabolism; D006360:Heat-Shock Proteins / Q000737:chemistry / Q000235:genetics / Q000502:physiology*; D006706:Homeostasis; D006801:Humans; D008107:Liver Diseases / Q000235:genetics; D008954:Models, Biological; D018832:Molecular Chaperones / Q000737:chemistry / Q000235:genetics / Q000502:physiology*; D009369:Neoplasms / Q000235:genetics / Q000378:metabolism; D017510:Protein Folding; D021381:Protein Transport / Q000502:physiology; D015398:Signal Transduction / Q000502:physiology; D013132:Spinocerebellar Degenerations / Q000235:genetics; D013577:Syndrome
D000091342:Endoplasmic Reticulum Chaperone BiP; D006360:Heat-Shock Proteins; D018832:Molecular Chaperones; D002118:Calcium