Rabbit Polyclonal LIPF Antibody

The lysosomal acid lipase (LIPF) antibody is a tool used to detect and study the LIPF enzyme, a key protein encoded by the LIPF gene. LIPF, also known as lysosomal acid lipase (LAL), is responsible for hydrolyzing cholesterol esters and triglycerides within lysosomes, facilitating lipid metabolism. Deficiencies in LAL activity due to LIPF mutations cause rare autosomal recessive disorders such as Wolman disease and cholesteryl ester storage disease (CESD), characterized by lysosomal lipid accumulation, hepatosplenomegaly, and multi-organ dysfunction. Antibodies targeting LIPF are critical for investigating these conditions, enabling researchers to quantify enzyme expression, assess tissue distribution, and validate experimental models. Clinically, LIPF antibodies aid in diagnosing LAL deficiency through immunohistochemistry or immunoblotting, complementing genetic and enzymatic activity assays. They also support therapeutic development, including monitoring responses to enzyme replacement therapies like sebelipase alfa. Both monoclonal and polyclonal LIPF antibodies are utilized, with applications spanning basic research, diagnostic workflows, and drug evaluation. As LAL's role extends beyond lipid metabolism to potential involvement in inflammation and metabolic syndromes, these antibodies remain vital for unraveling its pathophysiological mechanisms and therapeutic targets.