Gene: PDHA1
Official Full Name: pyruvate dehydrogenase E1 subunit alpha 1provided by HGNC
Gene Summary: The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial multienzyme complex that catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and provides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle. The PDH complex is composed of multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). The E1 enzyme is a heterotetramer of two alpha and two beta subunits. This gene encodes the E1 alpha 1 subunit containing the E1 active site, and plays a key role in the function of the PDH complex. Mutations in this gene are associated with pyruvate dehydrogenase E1-alpha deficiency and X-linked Leigh syndrome. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Mar 2010]
Catalog Number | Product Name | Species | Gene | Passage ratio | Mycoplasma testing | Price |
---|---|---|---|---|---|---|
KO01367 | PDHA1 Knockout cell line (HeLa) | Human | PDHA1 | 1:3~1:6 | Negative | Online Inquiry |
KO08224 | PDHA1 Knockout cell line (HCT 116) | Human | PDHA1 | 1:2~1:4 | Negative | Online Inquiry |
KO08225 | PDHA1 Knockout cell line (A549) | Human | PDHA1 | 1:3~1:4 | Negative | Online Inquiry |
KO39170 | PDHA1 Knockout cell line (HEK293) | Human | PDHA1 | 1:3~1:6 | Negative | Online Inquiry |
PDHA1 Gene Knockout Cell Lines represent a significant advancement in the realm of molecular biology and genetic research. These cell lines have been engineered to specifically lack the pyruvate dehydrogenase E1 alpha subunit (PDHA1), an essential component of the multi-enzyme complex pivotal for the conversion of pyruvate to acetyl-CoA. This knockout allows researchers to study the metabolic consequences of PDHA1 deficiency, particularly its implications in disorders such as lactic acidosis and mitochondrial dysfunction, which are closely linked to cellular energy metabolism and various pathophysiological conditions.
The key mechanism by which PDHA1 gene knockout exerts its function lies in the interruption of aerobic respiration and the resultant shift in metabolic pathways. By utilizing these cell lines, scientists can elucidate the compensatory metabolic adjustments and assess the impact on cellular processes including glycolysis, oxidative phosphorylation, and overall ATP production. Such insights are invaluable for advancing our understanding of metabolic diseases, enhancing therapeutic strategies, and developing new interventions targeting mitochondrial-related disorders.
The scientific importance of PDHA1 Gene Knockout Cell Lines is highlighted by their diverse applications in both research and clinical settings. They serve as an indispensable tool for studying the basic principles of cellular metabolism and provide a platform for drug screening and development, especially for compounds aimed at restoring metabolic balance in PDHA1-deficient conditions.
One of the standout features of our PDHA1 Gene Knockout Cell Lines is their unparalleled reliability and reproducibility compared to traditional models. While other cell lines may exhibit variable metabolic profiles, our cell lines offer a consistent background for experimentation, enabling researchers to draw precise conclusions and reducing variability in data interpretation. Moreover, their potential for gene editing and metabolic profiling makes them a superior choice for cutting-edge research.
The value of PDHA1 Gene Knockout Cell Lines to researchers, clinicians, and pharmaceutical developers cannot be overstated. They simplify complex investigations into mitochondrial function, expedite the search for therapeutic targets, and may ultimately contribute to the development of innovative treatments.
Our company is committed to advancing scientific discovery through high-quality biological products. With our expertise in cellular models and a focus on innovation, we provide researchers with the tools they need to push the boundaries of knowledge in metabolic research and beyond.
Please note that all services are for research use only. Not intended for any clinical use.
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