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Pyruvate Dehydrogenase Complex, Component X Proteins (PDHX)

The pyruvate dehydrogenase (PDH) complex is located in the mitochondrial matrix and catalyzes the conversion of pyruvate to acetyl coenzyme A. Additionally we are shipping Pyruvate Dehydrogenase Complex, Component X Antibodies (48) and Pyruvate Dehydrogenase Complex, Component X Kits (1) and many more products for this protein.

list all proteins Gene Name GeneID UniProt
PDHX 8050 O00330
PDHX 27402 Q8BKZ9
Rat PDHX PDHX 311254  
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Top Pyruvate Dehydrogenase Complex, Component X Proteins at antibodies-online.com

Showing 5 out of 5 products:

Catalog No. Origin Source Conjugate Images Quantity Supplier Delivery Price Details
HOST_Escherichia coli (E. coli) Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 29 to 34 Days
$4,331.68
Details
HOST_Escherichia coli (E. coli) Mouse His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 29 to 34 Days
$4,331.68
Details
HOST_Wheat germ Human GST tag 10 μg Log in to see 9 Days
$405.71
Details
Yeast Cow His tag   1 mg Log in to see 56 to 66 Days
$3,256.00
Details
HOST_Human Human Un-conjugated   20 μg Log in to see 9 to 11 Days
$785.40
Details

PDHX Proteins by Origin and Source

Origin Expressed in Conjugate
Human , ,
,
Mouse (Murine)

More Proteins for Pyruvate Dehydrogenase Complex, Component X (PDHX) Interaction Partners

Human Pyruvate Dehydrogenase Complex, Component X (PDHX) interaction partners

  1. We propose testing for the R446* mutation in PDHX as a rapid first screening in Roma infants with metabolic acidosis.

  2. MiR (show MLXIP Proteins)-26a regulates glucose metabolism of colorectal cancer cells by direct targeting PDHX.

  3. New mutation in PDHX gene found in two unrelated patients with Pyruvate dehydrogenase (show PDP Proteins) deficiency.

  4. genetic association with systemic lupus erythematosus to a haplotype between PDHX and CD44 (show CD44 Proteins) was established.

  5. determination that PDH (show PDP Proteins) and complex III exist at a steady-state ratio of 1:100, 1:128 and 1:202 in HeLa cell extracts, fibroblast mitochondria and heart tissue mitochondria, respectively

  6. model of the pyruvate dehydrogenase (show PDP Proteins) complex formed by E2 and E2 plus the E3-binding protein and binding of the E1 and E3 components

  7. specificity of pairing for human E3BP with E3 from its subcomplex structure to be most likely due to conformational rigidity of the binding fragment of the E3-binding domain of E3BP and its exquisite amino acid match with the E3 target interface

  8. A cluster of disease-causing E3 mutations located near the center of the E3BD/E3 interface prevents the efficient recruitment of these E3 variants by E3BP into the PDC (show PNKD Proteins), leading to the dysfunction of the PDC (show PNKD Proteins) catalytic machine.

  9. These data provide an additional case of E3BP deficiency with a unique and previously unreported deletion in the PDHX gene.

  10. Despite the presence of antibodies reactive with PDC (show PNKD Proteins)-E3BP in the majority of primary biliary cirrhosis (PBC (show DLAT Proteins)) patients this self-protein is not a dominant T-cell autoantigen in PBC (show DLAT Proteins).

Mouse (Murine) Pyruvate Dehydrogenase Complex, Component X (PDHX) interaction partners

  1. T1a/podoplanin (show PDPN Proteins) is first expressed in prox 1 (show PROX1 Proteins)-positive lymphatic progenitor cells, with predominant localization in the luminal plasma membrane of lymphatic endothelial cells during later development.

  2. In islet cells both fat and glucose regulate PDK (show PDK2 Proteins) gene and protein expression and indicate that hyperglycemia and hyperlipidemia contribute to the decline in diabetic islet PDH (show PDP Proteins) activity by increasing mRNA and protein expression of PDK (show PDK2 Proteins).

Cow (Bovine) Pyruvate Dehydrogenase Complex, Component X (PDHX) interaction partners

  1. This study showed that the recombinant and bovine E2/E3BP cores bind E3s with a 2:1 stoichiometry, and propose that mammalian PDC (show PDC Proteins) comprises a heterogeneous population of assemblies incorporating a network of E3 cross-bridges above the core surface.

Pyruvate Dehydrogenase Complex, Component X (PDHX) Protein Profile

Protein Summary

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 names and symbols associated with PDHX

  • pyruvate dehydrogenase complex, component X (pdhx)
  • pyruvate dehydrogenase complex, component X (PDHX)
  • E3-binding protein (pdhX)
  • pyruvate dehydrogenase complex, component X (LOC100220979)
  • pyruvate dehydrogenase complex, component X (LOC100349815)
  • pyruvate dehydrogenase complex, component X (Pdhx)
  • pyruvate dehydrogenase protein X component-like (LOC517402)
  • Ac1164 protein
  • AI481367 protein
  • DDBDRAFT_0168431 protein
  • DDBDRAFT_0230192 protein
  • DDB_0168431 protein
  • DDB_0230192 protein
  • DLDBP protein
  • E3bp protein
  • MGC86218 protein
  • OPDX protein
  • PDHX protein
  • Pdx1 protein
  • proX protein
  • zgc:66110 protein
  • zgc:85977 protein

Protein level used designations for PDHX

pyruvate dehydrogenase complex, component X , pyruvate dehydrogenase protein X component, mitochondrial-like , dihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex , lipoyl-containing pyruvate dehydrogenase complex component X , pyruvate dehydrogenase complex, E3-binding protein subunit , pyruvate dehydrogenase complex, lipoyl-containing component X , pyruvate dehydrogenase protein X component, mitochondrial , dihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex (E3-binding protein) (proX) , E3-binding protein , E3BP , proX , pyruvate dehydrogenase protein X component

GENE ID SPECIES
393532 Danio rerio
423154 Gallus gallus
447247 Xenopus laevis
451120 Pan troglodytes
8618072 Dictyostelium discoideum AX4
100059260 Equus caballus
100220979 Taeniopygia guttata
100349815 Oryctolagus cuniculus
100379696 Xenopus (Silurana) tropicalis
100455315 Pongo abelii
100475970 Ailuropoda melanoleuca
100544023 Meleagris gallopavo
100586539 Nomascus leucogenys
8050 Homo sapiens
27402 Mus musculus
311254 Rattus norvegicus
475942 Canis lupus familiaris
517402 Bos taurus
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