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PDHX / Protein X / ProX

pyruvate dehydrogenase complex, component X

The pyruvate dehydrogenase (PDH) complex is located in the mitochondrial matrix and catalyzes the conversion of pyruvate to acetyl coenzyme A. The PDH complex thereby links glycolysis to Krebs cycle. The PDH complex contains three catalytic subunits, E1, E2, and E3, two regulatory subunits, E1 kinase and E1 phosphatase, and a non-catalytic subunit, E3 binding protein (E3BP). This gene encodes the E3 binding protein subunit; also known as component X of the pyruvate dehydrogenase complex. This protein tethers E3 dimers to the E2 core of the PDH complex. Defects in this gene are a cause of pyruvate dehydrogenase deficiency which results in neurological dysfunction and lactic acidosis in infancy and early childhood. This protein is also a minor antigen for antimitochondrial antibodies. These autoantibodies are present in nearly 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In PBC, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC eventually leads to cirrhosis and liver failure. Alternative splicing results in multiple transcript variants encoding distinct isoforms.

Gene Name: pyruvate dehydrogenase complex, component X
Synonyms: PDHX, DLDBP, E3BP, OPDX, ProX, Protein x, E3-binding protein
Target Sequences: NM_003477 NP_003468.2 O00330

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Products
Proteins (12)
Over-Expression Lysate (6)
Recombinant (6)
PDHX / Protein X / ProX (12)
No (12)
6His-SUMO, N-terminus (1)
GST, N-terminus (1)
His (1)
His-T7 (2)
Myc-DDK (Flag) (7)
Hepatitis B Virus (1)
Human (7)
Mouse (1)
293T Cells (3)
E. coli (4)
HEK 293 Cells (4)
Wheat Germ Extract (1)
Purified (2)
PDHX / Protein X / ProX Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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HEK 293 Cells
Myc-DDK (Flag)
51.3 kDa
100 µg/$494
PDHX / Protein X / ProX Protein - (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
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E. coli
6His-SUMO, N-terminus
32.6kDa
1 mg/$1,679; 100 µg/$598; 20 µg/$405
PDHX / Protein X / ProX Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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HEK 293 Cells
Myc-DDK (Flag)
29.91 kDa
100 µg/$494
PDHX / Protein X / ProX Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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HEK 293 Cells
Myc-DDK (Flag)
53.9 kDa
100 µg/$494
PDHX / Protein X / ProX Protein
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E. coli
His
20 µg/$361; 100 µg/$739; 4 µg/$261
PDHX / Protein X / ProX Protein - Recombinant  Pyruvate Dehydrogenase Complex Component X By SDS-PAGE
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E. coli
His-T7
30.3 kDa
10 µg/$285; 50 µg/$467; 100 µg/$747; 200 µg/$932; 1 mg/$2,058; 500 µg/$1,590; 5 mg/$3,263; 2 mg/$2,246
PDHX / Protein X / ProX Protein - Recombinant  Pyruvate Dehydrogenase Complex Component X By SDS-PAGE
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E. coli
His-T7
30.2 kDa
10 µg/$280; 50 µg/$437; 100 µg/$700; 200 µg/$872; 1 mg/$1,978; 500 µg/$1,519; 5 mg/$3,053; 2 mg/$2,164
PDHX / Protein X / ProX Protein - Purified recombinant protein PDHX was analyzed by SDS-PAGE gel and Coomassie Blue Staining
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HEK 293 Cells
Myc-DDK (Flag)
53.9 kDa
20 µg/$1,107
PDHX / Protein X / ProX Protein - 12.5% SDS-PAGE Stained with Coomassie Blue.
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Wheat Germ Extract
GST, N-terminus
10 µg/$479; 25 µg/$670
PDHX / Protein X / ProX Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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293T Cells
Myc-DDK (Flag)
51.3 kDa
20 µg/$150
PDHX / Protein X / ProX Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
Select
293T Cells
Myc-DDK (Flag)
29.91 kDa
20 µg/$150
PDHX / Protein X / ProX Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
Select
293T Cells
Myc-DDK (Flag)
53.9 kDa
20 µg/$150
Viewing 1-12 of 12 product results


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For RESEARCH USE ONLY. Intended for use by laboratory professionals. Not intended for human diagnostic or therapeutic purposes.

The data on this page has been compiled from LifeSpan internal sources, the National Center for Biotechnology Information (NCBI), and The Universal Protein Resource (UniProt).