Anti-LONP1 Antibody

This rabbit polyclonal antibody targets LONP1 (Lon Peptidase 1, Mitochondrial), an ATP-dependent serine protease located in the mitochondrial matrix that mediates selective protein turnover and supports mitochondrial protein quality control. The reagent is supplied as 100 µl of unconjugated antiserum from Antibodies.com, validated for Western blot and immunohistochemistry on human and mouse samples.

LONP1 acts as a central effector of the mitochondrial unfolded protein response (UPRmt) and contributes to mtDNA copy number regulation and oxidative stress adaptation. Loss-of-function mutations in LONP1 cause CODAS syndrome, a multi-system developmental disorder, while altered LONP1 expression has been reported in several cancer types and in models of neurodegeneration. Researchers studying mitochondrial proteostasis, mtDNA maintenance, or disease-linked changes in this protease rely on the antibody to detect endogenous LONP1 in whole-cell lysates, enriched mitochondrial fractions, and fixed tissue sections.

As a polyclonal reagent raised in rabbit and unconjugated for flexible secondary-antibody pairing, the antibody fits standard immunoblotting and chromogenic or fluorescent IHC workflows. Reactivity has been confirmed against human and mouse samples; performance on other species has not been formally validated. The 100 µl format supports multiple experiments when the working stock is divided into single-use aliquots and stored refrigerated to limit freeze-thaw cycles and preserve binding activity over time.