SPCS2 is a 226 amino acid multi-pass membrane protein that localizes to both the microsome and the endoplasmic reticulum (ER), and belongs to the SPCS (signal peptidase complex subunit) family. Existing as a component of the microsomal signal peptidase complex which consists of five members, SPCS2 removes signal peptides from nascent proteins as they are translocated into the lumen of the ER. The gene encoding SPCS2 is located on human chromosome 11, which houses over 1,400 genes and comprises nearly 4% of the human genome. Jervell and Lange-Nielsen syndrome, Jacobsen syndrome, Niemann-Pick disease, hereditary angioedema and Smith-Lemli-Opitz syndrome are associated with defects in genes that maps to chromosome 11.