Fig. (4).

Schematic illustration of intracellular sorting of ABCC11 WT and proteasomal degradation of the R180 (Arg180) variant in secretory cells of the ceruminous apocrine gland. De novo synthesized ABCC11 WT is N-linked glycosylated at Asn838 and Asn844 in the ER, further processed in the Golgi apparatus, and destined for membrane of intracellular granules and vacuoles. Ceruminous components are thought to be transported by ABCC11 WT and sequestrated in intracellular granules and vacuoles. SNP variant R180 lacking N-linked glycosylation is recognized as misfolded proteins in the ER and readily undergo ubiquitination and proteasomal degradation (ERAD pathway). ER, endoplasmic reticulum; ERAD, ER-associated degradation.