A central idea in systems engineering is that complex systems are built by assembling components. System designers deal with a large variety of components, each having different characteristics, from a large variety of viewpoints, each highlighting different dimensions of a system. A central problem is the meaningful composition of heterogeneous components to ensure their correct interoperation...

Hek-293 cell line presents good production platform for recombinant therapeutic proteins, however little is known about the components that contribute to the cellular control of recombinant protein production. In this study, we generated a Hek-293 producing recombinant factor VIII (FVIII) and we evaluated the immunoglobulin-binding protein (BiP) and phytanoil-CoA α-hydroxylase (PAHX) expression...

Secretory proteins are cotranslationally translocated across the mammalian ER membrane through an aqueous pore in the translocon while the permeability barrier is maintained by a tight ribosome-membrane junction. The lumenal end of the pore is also blocked early in translocation. Extraction of soluble lumenal proteins from microsomes and reconstitution with purified proteins demonstrate, by flu...

Simian virus 40 (SV40) is a nonenveloped DNA virus that traffics through the endoplasmic reticulum (ER) en route to the nucleus, but the mechanisms of capsid disassembly and ER exit are poorly understood. We conducted an unbiased RNA interference screen to identify cellular genes required for SV40 infection. SV40 infection was specifically inhibited by up to 50-fold by knockdown of four differe...

Coordination languages simplify design and development of concurrent systems. Particularly, exogenous coordination languages, like BIP and Reo, enable system designers to express the interactions among components in a system explicitly. In this paper we establish a formal relation between BI(P) (i.e., BIP without the priority layer) and Reo, by defining transformations between their semantic mo...

gate that guards the translocon is opened and closed by ATP cycles that also give the same protein chaperone activity, as shown on page 389 by Alder et al. The translocon is an aqueous pore in the ER membrane through which secreted proteins pass during translation. To prevent the unwanted passage of ions, unused pores are plugged on the lumen side by the action of BiP. Within the ER, BiP is als...

This thesis was made to get a deeper understanding of how chaperones interact with unstable, aggregation prone, misfolded proteins involved in human disease. Over the last two decades, there has been much focus on misfolding diseases within the fields of biochemistry and molecular biotechnology research. It has become obvious that proteins that misfold (as a consequence of a mutation or outer f...