Monday 9 June 2003
The endoplasmic reticulum (ER) is an extensive three-dimensional network that stretches from the inner nuclear envelope to the cell cortex with a single, continuous membrane and a single, continuous lumen. The ER contains specialized regions that carry out unique functions.
smooth endoplasmic reticulum (SER)
rough endoplasmic reticulum (RER)
endoplasmic reticulum stress
- RNA translation diseases
- Wolcott-Rallison syndrome
endoplasmic reticulum retention
- alpha1-anti-trypsin deficiency (alpha1AT deficiency) variants exhibit a selective defect in migration of this secretory protein from the endoplasmic reticulum (ER) to Golgi apparatus.
- This is most marked for the PiZ polypeptide, attributable to a single amino acid substitution of Glu342 to Lys342. The mutant polypeptide (α1AT-Z) is abnormally folded, and polymerizes, causing its retention in the ER. All individuals with the PiZZ genotype accumulate α1AT-Z in the ER of hepatocytes.
reticulum endoplasmic stress
ER protein transport
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