SIL1 Homolog (SIL1)

SIL1 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.

The deduced 461-amino acid protein contains an N-terminal ER targeting sequence, 2 putative N-glycosylation sites, and a C-terminal ER retention signal. BAP mRNA contains 2 polyadenylation sequences. BAP shares 29% homology with Hsp70-binding protein-1 (HSPBP1), which inhibits the ATPase activity of Hsp70.