Home / BCKDHA Knockout Cell Lines

BCKDHA Knockout Cell Lines

Gene: BCKDHA

Official Full Name: branched chain keto acid dehydrogenase E1 subunit alphaprovided by HGNC

Gene Summary: The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Sep 2009]

Get A Quote
Products Background

Products

Catalog Number Product Name Species Gene Passage ratio Mycoplasma testing Price
KO38836 BCKDHA Knockout cell line (HeLa) Human BCKDHA 1:3~1:6 Negative Online Inquiry
KO38837 BCKDHA Knockout cell line (HCT 116) Human BCKDHA 1:2~1:4 Negative Online Inquiry
KO38838 BCKDHA Knockout cell line (HEK293) Human BCKDHA 1:3~1:6 Negative Online Inquiry
KO38839 BCKDHA Knockout cell line (A549) Human BCKDHA 1:3~1:4 Negative Online Inquiry

Background

BCKDHA Gene Knockout Cell Lines are specifically engineered cellular models that lack the gene encoding the branched-chain ketoacid dehydrogenase alpha (BCKDHA) enzyme. This enzyme plays a crucial role in the catabolism of branched-chain amino acids (BCAAs) – valine, leucine, and isoleucine – by facilitating their conversion into acyl-CoA derivatives, which are essential for energy production and metabolic homeostasis. The knockout of BCKDHA allows researchers to investigate the pathways influenced by disrupted BCAA metabolism, providing insights into metabolic disorders and neurological conditions linked to aberrant branched-chain amino acid levels.

The primary mechanism of action involves the complete inactivation of the BCKDHA gene, effectively providing a controlled system for analyzing the physiological consequences of impaired BCAA metabolism. This model is invaluable for studying various pathological states, including maple syrup urine disease and possibly other metabolic syndromes, enabling a deeper understanding of the role that BCAAs play in cellular processes.

The scientific importance of BCKDHA Gene Knockout Cell Lines lies in their applicability in both research and therapeutic contexts. These cell lines serve as vital tools for drug discovery, metabolic engineering, and basic biological research, allowing scientists to develop targeted interventions for metabolic diseases. Their unique ability to mimic the metabolic derangements seen in certain human disorders provides a more accurate framework for in vitro studies.

Compared to traditional cell lines, which may not adequately represent the effects of BCKDHA deficiency, these genetically modified models offer enhanced specificity and relevance for researchers. Moreover, the accessibility and functionality of these knockout cell lines streamline experimentation and reduce time to insights, offering significant advantages over alternative models.

Researchers and clinicians targeting metabolic dysfunctions can rely on BCKDHA Gene Knockout Cell Lines to elucidate molecular mechanisms and to explore potential therapeutic avenues. By employing these advanced cell models, researchers can better understand disease etiology, leading to the development of more effective approaches to treatment.

At our company, we pride ourselves on delivering high-quality, precision-engineered biological products supported by extensive expertise in genetic modification techniques. We are committed to advancing research through innovative solutions that empower scientists and clinicians alike.

Please note that all services are for research use only. Not intended for any clinical use.

Get a free quote

If your question is not addressed through these resources, you can fill out the online form below and we will answer your question as soon as possible.

0

There is no product in your cart.