Recombinant Human Pyruvate dehydrogenase protein X component, mitochondrial (PDHX)

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Code CSB-EP017718HU
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  • (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
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Product Details

Purity
Greater than 85% as determined by SDS-PAGE.
Target Names
PDHX
Uniprot No.
Research Area
Cancer
Alternative Names
(Dihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex)(E3-binding protein)(E3BP)(Lipoyl-containing pyruvate dehydrogenase complex component X)(proX)
Species
Homo sapiens (Human)
Source
E.coli
Expression Region
54-501aa
Target Protein Sequence
GDPIKILMPSLSPTMEEGNIVKWLKKEGEAVSAGDALCEIETDKAVVTLDASDDGILAKIVVEEGSKNIRLGSLIGLIVEEGEDWKHVEIPKDVGPPPPVSKPSEPRPSPEPQISIPVKKEHIPGTLRFRLSPAARNILEKHSLDASQGTATGPRGIFTKEDALKLVQLKQTGKITESRPTPAPTATPTAPSPLQATAGPSYPRPVIPPVSTPGQPNAVGTFTEIPASNIRRVIAKRLTESKSTVPHAYATADCDLGAVLKVRQDLVKDDIKVSVNDFIIKAAAVTLKQMPDVNVSWDGEGPKQLPFIDISVAVATDKGLLTPIIKDAAAKGIQEIADSVKALSKKARDGKLLPEEYQGGSFSISNLGMFGIDEFTAVINPPQACILAVGRFRPVLKLTEDEEGNAKLQQRQLITVTMSSDSRVVDDELATRFLKSFKANLENPIRLA
Note: The complete sequence including tag sequence, target protein sequence and linker sequence could be provided upon request.
Mol. Weight
55.5 kDa
Protein Length
Full Length of Mature Protein
Tag Info
N-terminal 10xHis-tagged and C-terminal Myc-tagged
Form
Liquid or Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
Buffer
If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.
Reconstitution
We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20°C/-80°C. Our default final concentration of glycerol is 50%. Customers could use it as reference.
Troubleshooting and FAQs
Storage Condition
Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
Shelf Life
The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
Lead Time
3-7 business days
Notes
Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet & COA
Please contact us to get it.

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Target Background

Function
Required for anchoring dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide transacetylase (E2) core of the pyruvate dehydrogenase complexes of eukaryotes. This specific binding is essential for a functional PDH complex.
Gene References into Functions
  1. We propose testing for the R446* mutation in PDHX as a rapid first screening in Roma infants with metabolic acidosis. PMID: 25087164
  2. MiR-26a regulates glucose metabolism of colorectal cancer cells by direct targeting PDHX. PMID: 24935220
  3. New mutation in PDHX gene found in two unrelated patients with Pyruvate dehydrogenase deficiency. PMID: 22766002
  4. genetic association with systemic lupus erythematosus to a haplotype between PDHX and CD44 was established. PMID: 21194677
  5. determination that PDH 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 PMID: 12372595
  6. model of the pyruvate dehydrogenase complex formed by E2 and E2 plus the E3-binding protein and binding of the E1 and E3 components PMID: 14638692
  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 PMID: 16263718
  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, leading to the dysfunction of the PDC catalytic machine. PMID: 16442803
  9. These data provide an additional case of E3BP deficiency with a unique and previously unreported deletion in the PDHX gene. PMID: 16566017
  10. Despite the presence of antibodies reactive with PDC-E3BP in the majority of primary biliary cirrhosis (PBC) patients this self-protein is not a dominant T-cell autoantigen in PBC. PMID: 16629643
  11. PDHX-assisted photosensitization with rose Bengal induces structural and functional alteration of mitochondria in HeLa cells. PMID: 17024456

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Involvement in disease
Pyruvate dehydrogenase E3-binding protein deficiency (PDHXD)
Subcellular Location
Mitochondrion matrix.
Protein Families
2-oxoacid dehydrogenase family
Database Links

HGNC: 21350

OMIM: 245349

KEGG: hsa:8050

STRING: 9606.ENSP00000227868

UniGene: Hs.502315

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