ELK Biotechnology PCSK9 rabbit pAb

PCSK9 rabbit pAb

제품 정보

세포 내 위치 (Cellular Localization)

Cytoplasm, Secreted, Endosome, Lysosome, Cell surface, Endoplasmic reticulum, Golgi apparatus.
Autocatalytic cleavage is required for transport from the endoplasmic reticulum to the Golgi apparatus and for secretion of the mature protein.
Localizes to the endoplasmic reticulum in the absence of LDLR and colocalizes to the cell surface and to the endosomes/lysosomes in the presence of LDLR.
Sorting to the cell surface and endosomes is required to fully promote LDLR degradation.

Background

• Cofactor: Calcium
• Disease association: Defects in PCSK9 cause familial hypercholesterolemia 3 (FH3) [MIM:603776], inherited in an autosomal dominant manner.
• Enzyme regulation: Inhibited by EGTA.
• Function: Implicated in cortical neuron differentiation and cholesterol homeostasis.
• PTM: The soluble zymogen undergoes autocatalytic intramolecular processing in the endoplasmic reticulum, resulting in cleavage of its propeptide, which remains associated with the secreted enzyme.
• Similarity: Belongs to the peptidase S8 family; contains one peptidase S8 domain.
• Subunit: The precursor protein, but not the mature protein, may form multimers.
• Tissue specificity: Expressed in neuro-epithelioma, colon carcinoma, hepatic and pancreatic cell lines, and Schwann cells.