MMUT抗体 新品

MMUT antibody targets methylmalonyl-CoA mutase (MCM), a mitochondrial enzyme encoded by the *MMUT* gene, which plays a critical role in catabolizing branched-chain amino acids, odd-chain fatty acids, and cholesterol derivatives. MCM facilitates the isomerization of methylmalonyl-CoA to succinyl-CoA, a key step in the mitochondrial Krebs cycle and energy production. Mutations in *MMUT* are linked to methylmalonic acidemia (MMA), a rare inherited metabolic disorder characterized by the accumulation of methylmalonic acid, leading to life-threatening complications such as metabolic acidosis, neurological impairment, and multi-organ dysfunction. MMUT antibodies are essential tools in research and diagnostics, enabling the detection of MCM protein expression levels, localization, and potential dysfunction in patient samples or experimental models. These antibodies are widely used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to study disease mechanisms, assess gene therapy outcomes, or validate cellular models of MMA. Additionally, they aid in distinguishing MMA subtypes caused by *MMUT* mutations from those arising from defects in other genes (e.g., *MMAA*, *MMAB*), informing targeted therapeutic strategies.