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![Pyruvate Dehydrogenase E1 beta subunit Recombinant Rabbit Monoclonal Antibody [JM66-33] — image 1 of 3](https://cdn.genprice.uk/products/992/68dd1c8c061270361ea81a6d/imageurl/et1705-57.webp)
Product details
Catalog number ET1705-57
Price for ET1705-57
Size / count 100 µL
The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO. It contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). Defects in PDHB are a cause of pyruvate dehydrogenase E1 component deficiency (PDHE1 deficiency). PDHE1 deficiency is the most common enzyme defect in patients with primary lactic acidosis. It is associated with variable clinical phenotypes ranging from neonatal death to prolonged survival complicated by developmental delay, seizures, ataxia, apnea, and in some cases to an X-linked form of Leigh syndrome (LS) (Leigh encephalomyelopathy).
The catalog number for Pyruvate Dehydrogenase E1 beta subunit Recombinant Rabbit Monoclonal Antibody [JM66-33] is ET1705-57.
Pyruvate Dehydrogenase E1 beta subunit Recombinant Rabbit Monoclonal Antibody [JM66-33] is available in the following size(s): 100 µL.
Pyruvate Dehydrogenase E1 beta subunit Recombinant Rabbit Monoclonal Antibody [JM66-33] is currently made to order / not held in immediate stock. Contact us for lead time.
The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO. It contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). Defects in PDHB are a cause of pyruv
Animal-origin-free status, where applicable, is noted on the datasheet under formulation details.
If you’re outside the EU, UK, or USA, you’re responsible for customs clearance and any related taxes or fees. We provide the standard import documents, such as the Commercial Invoice, Packing List, and Airway Bill, in advance, so let us know early if your customs office needs anything additional for this specific product.
The formulation or storage buffer is listed on the product datasheet. If you require a carrier-free or custom formulation, contact us before ordering, as this may involve a made-to-order production timeline.
