Anti-PEX19 / Peroxisomal biogenesis factor 19

Adding 0.2 ml of distilled water will yield a concentration of 500 ug/ml. PEX19 antibody detects Peroxisomal biogenesis factor 19, a cytosolic chaperone and receptor required for peroxisome membrane assembly. The UniProt recommended name is Peroxisomal biogenesis factor 19 (PEX19). This protein is essential for the import and insertion of peroxisomal membrane proteins (PMPs), ensuring proper peroxisome formation, maintenance, and function.Functionally, PEX19 antibody recognizes a 299-amino-acid protein that binds newly synthesized PMPs in the cytosol, shielding them from aggregation and misfolding. PEX19 delivers these PMPs to the peroxisomal membrane by interacting with the docking protein PEX3, which anchors them into the membrane bilayer. This process is vital for peroxisomal membrane protein biogenesis, division, and inheritance. PEX19 functions both as a chaperone and an import receptor, playing a dual role in organelle biogenesis.The PEX19 gene is located on chromosome 1q22 and encodes a soluble protein that cycles between cytosolic and peroxisomal compartments. It contains a C-terminal farnesylation motif that anchors it transiently to membranes, enhancing interaction with peroxisomal receptors. Mutations in PEX19 cause peroxisome biogenesis disorder type 14B (Zellweger spectrum disorder), resulting in defective peroxisome formation and metabolic dysfunction characterized by accumulation of very-long-chain fatty acids and bile acid intermediates.In healthy cells, PEX19 interacts with multiple peroxins, including PEX3 and PEX16, forming a network that ensures correct targeting and integration of PMPs. Its function is tightly coordinated with matrix protein import, supporting peroxisomal maintenance and proliferation. Beyond peroxisome assembly, PEX19 may influence lipid metabolism and antioxidant defense through its role in peroxisomal homeostasis. Dysregulation of PEX19 expression contributes to oxidative stress and metabolic imbalance.PEX19 antibody is used in cell biology, biochemistry, and metabolism research to study peroxisomal formation and dynamics. It supports applications such as immunoblotting, immunofluorescence, and organelle isolation to evaluate peroxisome abundance, protein import, and morphology. The antibody is especially valuable for identifying peroxisomal defects in metabolic and neurodegenerative diseases. In hepatocytes and fibroblasts, PEX19 localization provides a sensitive indicator of peroxisome assembly status.Structurally, PEX19 contains multiple tetratricopeptide repeat (TPR)-like motifs that mediate binding to PMPs. Its N-terminal region recognizes hydrophobic transmembrane segments, while the C-terminal farnesylation site facilitates membrane targeting. NSJ Bioreagents provides PEX19 antibody reagents validated for use in peroxisome biogenesis, organelle dynamics, and lipid metabolism research.