RCC1L抗体 新品

The RCC1L antibody targets proteins associated with the Regulator of Chromosome Condensation 1 (RCC1)-like family, which shares structural homology with RCC1. a critical guanine nucleotide exchange factor (GEF) for the small GTPase Ran. RCC1L proteins, such as RCC1L (also termed WBSCR16 or HB1), are implicated in diverse cellular processes, including mitochondrial function, ribosome biogenesis, and chromatin remodeling. RCC1L localizes to mitochondria and interacts with components of the mitochondrial ribosome, suggesting a role in mitochondrial translation or respiratory chain assembly. Dysregulation of RCC1L has been linked to neurodevelopmental disorders and mitochondrial diseases. Antibodies against RCC1L are primarily used in research to investigate its expression patterns, subcellular localization, and interactions via techniques like Western blotting, immunofluorescence, and immunoprecipitation. These tools have helped uncover its potential involvement in pathologies like Williams-Beuren syndrome (due to its proximity to the chromosomal deletion region) and cancer, where mitochondrial dysfunction is a hallmark. Commercial RCC1L antibodies are typically raised in rabbits or mice, with validation in knockout models to ensure specificity. Ongoing studies aim to clarify its molecular mechanisms and therapeutic relevance.