BCS1L抗体—艾普蒂

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     The image is immunohistochemistry of paraffin-embedded Human colorectal cancer tissue using P10444(BCS1L Antibody) at dilution 1/200. (Original magnification: ×200)    

  
  

The BCS1L antibody targets the BCS1L (BCS1 Homolog, Ubiquinol-Cyanide Reductase Complex Chaperone) protein, a critical mitochondrial chaperone involved in assembling respiratory chain Complex III (ubiquinol-cytochrome c reductase). BCS1L facilitates the insertion of the Rieske iron-sulfur subunit into Complex III, essential for electron transport and oxidative phosphorylation. Mutations in the BCS1L gene are linked to severe mitochondrial disorders, including GRACILE syndrome (growth retardation, aminoaciduria, cholestasis, iron overload, lactic acidosis, and early death) and Björnstad syndrome (characterized by sensorineural hearing loss and pili torti).

BCS1L antibodies are widely used in research to study mitochondrial dysfunction, protein expression patterns, and disease mechanisms. They are applied in techniques like Western blotting, immunofluorescence, and immunohistochemistry to visualize BCS1L localization in tissues or cultured cells. These antibodies also aid in diagnosing genetic disorders by detecting abnormal protein levels or mutations in clinical samples. Recent studies explore BCS1L's role beyond mitochondrial respiration, including its potential involvement in cellular stress responses and cancer metabolism. Commercial BCS1L antibodies are typically raised in rabbits or mice, with validation across species like human, mouse, and rat, supporting cross-disciplinary research in biochemistry, genetics, and translational medicine.