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* A set of membranes that form a single functional and developmental unit, either being
connected directly, or exchanging material through vesicle transport.

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Figure 12-34a Molecular Biology of the Cell (© Garland Science 2008)
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Cotranslational targeting:
As they emerge from the ribosome, signal sequences are bound by a signal recognition
particle (SRP).
SRPs consist of six polypeptides and a small cytoplasmic RNA (SRP RNA).
The SRP binds the ribosome and the signal sequence, inhibiting further translation.
The entire complex binds to an SRP receptor on the rough ER membrane.
The SRP is then released, and the ribosome binds to a membrane channel or translocon.
The signal sequence is inserted into the translocon, and translation resumes.
The signal sequence is cleaved by signal peptidase and released into the ER lumen.

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*en bloc : as a unit, all together

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A protein folding sensor assesses the folded glycoproteins; if correctly folded they exit
the ER.
If not folded correctly, the folding sensor will add back a glucose residue, allowing it to
cycle back to calnexin or calreticulin for another attempt at folding.
A severely misfolded glycoprotein is recognized by EDEM1, which removes mannose
residues.
The protein is returned to the cytosol through a ubiquitin ligase complex where it is
marked by ubiquitylation and degraded in a proteasome.

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Cell5e-Fig-10-15-0.jpg

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Cell5e-Fig-08-25-0.jpg

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Proteoglycans contain GAG chains.
*Remember that ceramides are synthesized in the sER.

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*GAGs (glycosaminoglycans) are long unbranched polysaccharides consisting of a
repeating disaccharide unit. The repeating unit consists of a hexose (six-carbon sugar) or
a hexuronic acid, linked to a hexosamine (six-carbon sugar containing nitrogen).

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*GlcNAc = N-Acetylglucosamine

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Aka mucolipidosis II

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Cell5e-Fig-10-37-1.jpg

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