KLHL1 (Kelch-like protein 1) is a member of the Kelch-like family, characterized by its BTB (Broad-Complex, Tramtrack, and Bric-a-brac) domain and Kelch repeat motifs. These structural features enable KLHL1 to act as a substrate-specific adaptor for Cullin 3 (CUL3)-based E3 ubiquitin ligase complexes, facilitating the targeted ubiquitination and subsequent degradation of proteins. KLHL1 is predominantly expressed in the nervous system, particularly in the brain and cerebellum, where it plays roles in neuronal development, synaptic plasticity, and ion channel regulation. Studies suggest its involvement in modulating voltage-gated calcium channels (e.g., Cav2.1) and potassium channels, impacting neuronal excitability and signaling.
Antibodies targeting KLHL1 are essential tools for investigating its expression, localization, and molecular interactions. They are widely used in techniques such as Western blotting, immunohistochemistry (IHC), and immunoprecipitation (IP) to study KLHL1's role in physiological and pathological contexts. For instance, KLHL1 dysfunction has been linked to neurological disorders, including spinocerebellar ataxia and epilepsy, making its antibodies valuable for mechanistic research. Additionally, KLHL1's potential involvement in cancer progression, via regulation of ubiquitination pathways, underscores its relevance in oncological studies. Commercial KLHL1 antibodies are typically validated for specificity using knockout controls or siRNA-based knockdowns. Researchers must verify antibody performance in their experimental systems, as cross-reactivity with other KLHL family members (e.g., KLHL2. KLHL3) may occur due to structural similarities.