ABCB7抗体

The ABCB7 antibody is a crucial tool for studying the ATP-binding cassette subfamily B member 7 (ABCB7), a mitochondrial transmembrane protein involved in iron-sulfur (Fe-S) cluster biogenesis and cellular iron homeostasis. ABCB7 facilitates the transport of Fe-S clusters from mitochondria to the cytosol, a process vital for numerous enzymatic functions, including DNA repair and electron transport. Mutations in the ABCB7 gene are linked to X-linked sideroblastic anemia with ataxia (XLSA/A), a rare disorder characterized by defective heme synthesis and neurological impairment. The ABCB7 antibody enables the detection and localization of the ABCB7 protein in various tissues and cell lines, aiding research into its expression patterns under physiological and pathological conditions. It is widely used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to investigate ABCB7’s role in mitochondrial iron metabolism, oxidative stress responses, and diseases such as anemia, neurodegenerative disorders, and cancer. Recent studies also explore its potential interactions with other Fe-S cluster assembly proteins, offering insights into molecular mechanisms underlying iron-related pathologies. By providing specificity and reliability, the ABCB7 antibody remains essential for advancing understanding of mitochondrial function and iron regulation in health and disease.