Loading...

Skip to main content

Call us on + 1 (800) 604-9114 for more information about our products

Looking for specific datasheet Manual/COA/MSDS?
Request a Manual/COA/MSDS

Interested to get a quote about our products?
Request a Quote

PDHA1 cell lysate

Human PDHA1 Baculovirus-Insect cells Overexpression Lysate

Gene Names
PDHA1; PDHA; PHE1A; PDHCE1A
Applications
Western Blot
Synonyms
PDHA1; Human PDHA1 Baculovirus-Insect cells Overexpression Lysate; Human PDHA1/C54G1 (aa 30-390) Insect Cell Lysate (WB positive control); pyruvate dehydrogenase (lipoamide) alpha 1; Human PDHA Overexpression Lysate; Human PDHAD Overexpression Lysate; Human PDHCE1A Overexpression Lysate; Human PHE1A Overexpression Lysate; PDHA1 cell lysate
Ordering
For Research Use Only!
Host
Baculovirus-Insect cells
Form/Format
1x Sample Buffer (1x modified RIPA buffer+1x SDS loading buffer).
Modified RIPA Lysis Buffer: 50 mM Tris-HCl pH 7.4, 150 mM NaCl, 1mM EDTA, 1% Triton X-100, 0.1% SDS, 1% Sodium deoxycholate, 1mM PMSF.
Sequence Positions
598aa
Sequence
Phe30-Ser390
Applicable Applications for PDHA1 cell lysate
Western Blot (WB)
Application Notes
WB: Use at an assay dependent dilution.
Species
Human
Recommend Usage
1. Centrifuge the tube for a few seconds and ensure the pellet at the bottom of the tube.2. Re-dissolve the pellet using 200uL pure water and boil for 2-5 min.
Sequence Construction
A DNA sequence encoding the human PDHA1 (P08559-1) (Phe30-Ser390) was fused with the N-terminal polyhistidine-tagged GST tag at the N-terminus.
Preparation Method
Cell lysate was prepared by homogenization of the over-expressed cells in ice-cold modified RIPA Lysis Buffer with cocktail of protease inhibitors. Cell debris was removed by centrifugation. Protein concentration was determined by Bradford assay (protein assay, Microplate Standard assay). The cell lysate was boiled for 5 min in 1 x SDS loading buffer (50mM Tris-HCl pH 6.8, 12.5% glycerol, 1% sodium dodecylsulfate, 0.01% bromophenol blue) containing 5% b-mercaptoethanol, and lyophilized.
Preparation and Storage
Store at 4 degree C for up to twelve months from date of receipt. After re-dissolution, aliquot and store at -80 degree C for up to twelve months. Avoid repeated freeze-thaw cycles.
Samples are stable for up to twelve months from date of receipt.
Related Product Information for PDHA1 cell lysate
PDHA1, also known as C54G1, is an alpha subunit of pyruvate dehydrogenase. Pyruvate dehydrogenase, together with dihydrolipoamide acetyltransferase and lipoamide dehydrogenase, composes the pyruvate dehydrogenase (PDH) complex. The 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. PDHA1 plays a key role in the function of the PDH complex. Defects in PDHA1 can cause pyruvate dehydrogenase E1-alpha deficiency. Defects in PDHA1 also are the cause of X-linked Leigh syndrome (X-LS). X-LS is an early-onset progressive neurodegenerative disorder with a characteristic neuropathology consisting of focal, bilateral lesions in one or more areas of the central nervous system, including the brainstem, thalamus, basal ganglia, cerebellum, and spinal cord.

This Human PDHA1 overexpression lysate was created in Baculovirus-Insect cells and intented for use as a Western blot (WB) positive control. Purification of PDHA1 protein from the overexpression lysate was verified.
References
Quintana E. et al., 2010, Clin Genet. 77 (5): 474-82. Ah Mew N. et al., 2011, Pediatr Neurol. 45 (1): 57-9. Pinheiro A. et al., 2012, Gene. 506 (1): 173-8.

NCBI and Uniprot Product Information

NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
Molecular Weight
43,296 Da
NCBI Official Full Name
pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial isoform 1
NCBI Official Synonym Full Names
pyruvate dehydrogenase (lipoamide) alpha 1
NCBI Official Symbol
PDHA1
NCBI Official Synonym Symbols
PDHA; PHE1A; PDHCE1A
NCBI Protein Information
pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial; PDHE1-A type I; pyruvate dehydrogenase complex, E1-alpha polypeptide 1
UniProt Protein Name
Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
UniProt Gene Name
PDHA1
UniProt Synonym Gene Names
PHE1A
UniProt Entry Name
ODPA_HUMAN

NCBI Description

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]

Uniprot Description

PDHA1: a mitochondrial matrix enzyme that catalyzes the oxidative decarboxylation of pyruvate, producing acetyl-CoA and CO2. A key enzyme in controlling the balance between lipid and glucose oxidation depending on substrate availability. The pyruvate dehydrogenase (PDH) holoenzyme is a multi-enzyme complex (PDHC) that contains 20-30 copies of pyruvate decarboxylase tetramers (2 alpha:2 beta)(E1), 60 copies of dihydrolipoamide acetyltransferase (E2), six homodimers of dihydrolipoamide dehydrogenase (E3), plus E3 binding proteins. The activity of PDH is tightly regulated by phosphorylation. The phosphorylation of at least one of three specific serine residues in E1 subunit by PDHK inactivates the PDHC, while dephosphorylation by PDP restores its activity. Sites 1, 2, and 3 of PDHA1 are S293, S300, and S232, respectively. Four PDHK isoenzymes have been described, each with different site specificity: all four phosphorylate sites 1 and 2 but at different rates; for site 1 PDHK2 >PDHK4 >PDHK1 >PDHK3; for site 2, PDHK3> PDHK4 > PDHK2 > PDHK1. Only PDHK1 phosphorylates site 3. PDHA1 deficiency is the most common enzyme defect in patients with primary lactic acidosis.

Protein type: Carbohydrate Metabolism - citrate (TCA) cycle; Carbohydrate Metabolism - glycolysis and gluconeogenesis; Carbohydrate Metabolism - butanoate; Oxidoreductase; EC 1.2.4.1; Carbohydrate Metabolism - pyruvate; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine biosynthesis

Chromosomal Location of Human Ortholog: Xp22.1

Cellular Component: mitochondrion; mitochondrial matrix; pyruvate dehydrogenase complex; nucleus

Molecular Function: pyruvate dehydrogenase activity; pyruvate dehydrogenase (acetyl-transferring) activity

Biological Process: cellular metabolic process; acetyl-CoA biosynthetic process from pyruvate; glycolysis; tricarboxylic acid cycle; glucose metabolic process; regulation of acetyl-CoA biosynthetic process from pyruvate; pyruvate metabolic process

Disease: Pyruvate Dehydrogenase E1-alpha Deficiency

Research Articles on PDHA1

Similar Products

Product Notes

The PDHA1 pdha1 (Catalog #AAA8115404) is a Cell Lysate produced from Baculovirus-Insect cells and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is 598aa. AAA Biotech's PDHA1 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). WB: Use at an assay dependent dilution. Researchers should empirically determine the suitability of the PDHA1 pdha1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. The amino acid sequence is listed below: Phe30-Ser3 90. It is sometimes possible for the material contained within the vial of "PDHA1, Cell Lysate" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.

Precautions

All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.

Disclaimer

Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.

Item has been added to Shopping Cart

If you are ready to order, navigate to Shopping Cart and get ready to checkout.

Looking for a specific manual?
Request a Manual

Request more Information

Please complete the form below and a representative will contact you as soon as possible.

Request a Manual

Please complete the form below and a representative will contact you as soon as possible.

Request a Quote

Please complete the form below and a representative will contact you as soon as possible.