Sequence Alterations in the Carboxyl- Terminal Propeptide Do main - Osteogenesis Imperfecta - page 126

[83] Schroder M. Endoplasmic reticulum stress responses. Cell Mol

Life Sci 2008;65(6):862-94.

[88] Lisse TS, Thiele F, Fuchs H, et al. ER stress-mediated apoptosis

in a new mouse model of osteogenesis imperfecta. PLoS Genet
2008;4(2):e7. [84] Brodsky JL. The protective and destructive roles played by

molecular chaperones during ERAD (endoplasmic-reticulum-
associated degradation). Biochem J 2007;404(3):353-63.

[89] Forlino A, Tani C, Rossi A, et al. Differential expression of both

extracellular and intracellular proteins is involved in the lethal
or nonlethal phenotypic variation of BrtlIV, a murine model for
osteogenesis imperfecta. Proteomics 2007;7(11):1877-91.

[85] Bernales S, Schuck S, Walter P. ER-phagy: selective autophagy of

the endoplasmic reticulum. Autophagy 2007;3(3):285-7.

[86] Ishida Y, Yamamoto A, Kitamura A, et al. Autophagic elimination

of misfolded procollagen aggregates in the endoplasmic reticulum
as a means of cell protection. Mol Biol Cell 2009;20(11):2744-54.

[90] Cohen FE, Kelly JW. Therapeutic approaches to protein-misfold-

ing diseases. Nature 2003;426(6968):905-9.

[87] Fitzgerald J, Lamande SR, Bateman JF. Proteasomal degradation

of unassembled mutant type I collagen pro-alpha1(I) chains.
J Biol Chem 1999;274(39):27392-8.