Anti-Glucosidase 2 subunit beta antibody [EPR8046] - BSA and Azide free

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

Glucosidase 2 subunit beta also known as PRKCSH plays an important role in protein processing within the endoplasmic reticulum (ER). This protein forms part of the heterodimeric enzyme complex glucosidase II responsible for cleaving glucose molecules during glycoprotein maturation. With a molecular weight of about 60 kDa glucosidase 2 subunit beta is important for efficient N-linked glycosylation which is a fundamental process for protein folding and quality control. This protein localizes mainly to the ER where it contributes to proper cellular functions by aiding in the preparation of proteins for trafficking to their final destinations.

Biological function summary

Glucosidase 2 subunit beta is associated with protein maturation and quality control within the ER lumen. It is part of the glucosidase II complex which functions to trim glucose residues from N-linked oligosaccharides on nascent glycoproteins facilitating their proper folding and preventing misfolded proteins' accumulation. This activity ensures that only properly folded proteins progress through the secretory pathway while faulty proteins are targeted for degradation. Through these processes glucosidase 2 subunit beta supports cellular homeostasis and protein turnover.

Pathways

Glucosidase 2 subunit beta plays an influential role in the protein processing and ER-associated degradation (ERAD) pathways. The protein operates within these pathways to regulate glycoprotein folding impacting the unfolding protein response (UPR). It interacts with several proteins including calnexin and calreticulin which are key players in the glycoprotein folding process. Moreover the proper function of glucosidase 2 subunit beta in these pathways is necessary for maintaining the cellular stress response ultimately ensuring efficient cell function and survival.

Associated diseases and disorders

Defects in glucosidase 2 subunit beta have links to autosomal dominant polycystic liver disease (PCLD). Mutations in PRKCSH which encodes glucosidase 2 subunit beta can lead to the development of PCLD due to disrupted glycoprotein folding and ER stress-induced apoptosis. Additionally abnormalities in its function may indirectly connect to diabetes mellitus by affecting insulin receptor glycosylation and secretion processes. These relationships illustrate the broader impact of glucosidase 2 subunit beta on health and disease providing insight into potential therapeutic targets for treatment strategies.