PPOX Antibody (42J-6): sc-100577

PPOX Antibody (42J-6) is a mouse monoclonal IgG1 antibody that detects protoporphyrinogen oxidase (PPOX) in human samples through various applications including western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), immunohistochemistry with paraffin-embedded sections (IHCP), and enzyme-linked immunosorbent assay (ELISA). Protoporphyrinogen oxidase plays a crucial role as the penultimate enzyme in the heme biosynthetic pathway, facilitating the conversion of protoporphyrinogen IX to protoporphyrin IX through a 6-electron oxidation process. This enzymatic activity is vital for the production of heme, an essential component of hemoglobin and various cytochromes, which are critical for oxygen transport and cellular respiration. PPOX (42J-6) antibody targets a protein predominantly located in the inner membrane of mitochondria across multiple tissues, including the heart, brain, placenta, lung, liver, skeletal muscle, kidney, and pancreas, underscoring its importance in energy metabolism and cellular function. Genetic deficiencies in PPOX can lead to variegate porphyria, an autosomal dominant disorder that manifests with cutaneous photosensitivity and neurological symptoms, highlighting the significance of this protein in maintaining metabolic homeostasis. The rare homozygous variant of variegate porphyria is particularly severe, resulting in early childhood onset of photosensitization, skeletal abnormalities, and potential cognitive impairments, further emphasizing the critical nature of protoporphyrinogen oxidase in human health.

The preferred PPOX antibody is PPOX Antibody (C-12): sc-271768.