Translation of Journal of Cell Science

Prosser, D. C., et al.
"FFAT Rescues VAPA-Mediated Inhibition of ER-to-Golgi
Transport and VAPB-Mediated ER Aggregation."
Journal of Cell Science, vol. 121, no. 18, 19 June 2008, pp. 3052-3061.,
doi:10.1242/jcs.028696.

(Translated by Sohail Syed)

Significance

Amyotrophic lateral sclerosis (ALS8) has been linked to the P56S mutation of VAPA and VAPB. The over expression of these VAPs has been seen to cause ER aggregation and inhibit transport of proteins from the ER to the Golgi apparatus. With this aggregation and inhibition our cells can not function properly as it will not allow proper transport of necessary proteins to sustain cell life. But in order to counteract this it has been found that the FFAT motif helps rescue VAPs as it can encourage the transport of membrane proteins and decrease ER aggregation allow proper folding of proteins. With this being said, not much is known about how the FFAT motif does this. In order to explain this phenomena, 6 experiments were done as with the increase in knowledge of the inter-workings of the FFAT motif, a possible cure/help for ALS8 can be found.

Contents

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