
E3BP Antibody (C-2) is a mouse monoclonal IgG2a antibody that detects E3BP in mouse, rat, and human samples through applications such as western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). E3BP, also known as PDHX (Pyruvate dehydrogenase protein X component) or Lipoyl-containing pyruvate dehydrogenase complex component X, is a crucial 501 amino acid mitochondrial protein that plays a vital role in the proper functioning of the pyruvate dehydrogenase (PDH) complex. This complex serves as the primary link between glycolysis and the tricarboxylic acid (TCA) cycle by catalyzing the irreversible conversion of pyruvate into acetyl-CoA, a key metabolic intermediate. E3BP functions as an anchor for the E3 component to the E2 core of the PDH complex, a critical interaction necessary for maintaining the integrity and functionality of the entire enzyme complex. Deficiencies in the gene encoding E3BP can lead to severe metabolic disorders, including pyruvate dehydrogenase E3-binding protein deficiency, which presents with symptoms such as lactic acidosis, developmental delays, seizures, and other neurological impairments. Anti-E3BP antibody (C-2) is an essential tool for researchers studying mitochondrial function and related metabolic diseases, providing insights into the molecular mechanisms underlying these conditions.
Ordering Information
| Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
E3BP Antibody (C-2) | sc-393644 | 200 µg/ml | $322.00 | |||
E3BP Antibody (C-2): m-IgG Fc BP-HRP Bundle | sc-538268 | 200 µg Ab; 10 µg BP | $361.00 | |||
E3BP Antibody (C-2): m-IgGκ BP-HRP Bundle | sc-535845 | 200 µg Ab; 40 µg BP | $361.00 | |||
E3BP Antibody (C-2): m-IgG2a BP-HRP Bundle | sc-548251 | 200 µg Ab; 10 µg BP | $361.00 |