Glucosidase 2 subunit beta Mouse Monoclonal Antibody [A6H9] (HA600054)

UNIPROT #

P14314

PROTEIN NAME

Glucosidase 2 subunit beta

SYNONYMS

80K-H protein antibody;AGE-binding receptor 2 antibody;AGE-R2 antibody;G19P1 antibody;GLU2B_HUMAN antibody;Glucosidase 2 subunit beta antibody;Glucosidase II beta subunit antibody;Glucosidase II subunit beta antibody;Hepatocystin antibody;PCLD antibody;PKCSH antibody;PLD1 antibody;PRKCSH antibody;Protein kinase C substrate 60.1 kDa protein heavy chain antibody;Protein kinase C substrate 80 Kda protein antibody;Protein kinase C substrate 80K-H antibody;Protein kinase C substrate, 80 Kda protein antibody

SUBCELLULAR LOCATION

Endoplasmic reticulum.

FUNCTION

Regulatory subunit of glucosidase II that cleaves sequentially the 2 innermost alpha-1,3-linked glucose residues from the Glc(2)Man(9)GlcNAc(2) oligosaccharide precursor of immature glycoproteins. Required for efficient PKD1/Polycystin-1 biogenesis and trafficking to the plasma membrane of the primary cilia (By similarity). This gene encodes the beta-subunit of glucosidase II, an N-linked glycan-processing enzyme in the endoplasmic reticulum. The encoded protein is an acidic phosphoprotein known to be a substrate for protein kinase C. Mutations in this gene have been associated with the autosomal dominant polycystic liver disease. Alternative splicing results in multiple transcript variants.