
This gene encodes a resident endoplasmic reticulum (ER)| N-linked glycoprotein with an N-terminal ER targeting sequence| 2 putative N-glycosylation sites| and a C-terminal ER retention signal. This protein functions as a nucleotide exchange factor for another unfolded protein response protein. Mutations in this gene have been associated with Marinesco-Sjogren syndrome. Alternate transcriptional splice variants have been characterized.

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