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Pyruvate Dehydrogenase Complex Deficiency Disease: Disease Bioinformatics

Research of Pyruvate Dehydrogenase Complex Deficiency Disease has been linked to Acidosis, Acidosis, Lactic, Leigh Disease, Acidemia, Inborn Errors Of Metabolism. The study of Pyruvate Dehydrogenase Complex Deficiency Disease has been mentioned in research publications which can be found using our bioinformatics tool below. Researched pathways related to Pyruvate Dehydrogenase Complex Deficiency Disease include Pyruvate Oxidation, Brain Development, Transport, Pathogenesis, Excretion. These pathways complement our catalog of research reagents for the study of Pyruvate Dehydrogenase Complex Deficiency Disease including antibodies and ELISA kits against PYRUVATE DECARBOXYLASE, TPP, ALDH9A1, BCHE, CSF2.

Top Research Reagents

We have 1733 products for the study of Pyruvate Dehydrogenase Complex Deficiency Disease that can be applied to Chromatin Immunoprecipitation, Chromatin Immunoprecipitation (ChIP), Flow Cytometry, Immunocytochemistry/ Immunofluorescence, Immunohistochemistry, Western Blot from our catalog of antibodies and ELISA kits.

Dihydrolipoamide Dehydrogenase/DLD Antibody
NBP1-31302
Western Blot: Dihydrolipoamide Dehydrogenase/DLD Antibody [NBP1-31302] - Wild-type (WT) and DLD knockout (KO) HeLa cell extracts (30 ug) were separated by 10% SDS-PAGE, and the membrane was blotted with DLD antibody diluted at 1:500. HRP-conjugated anti-rabbit IgG antibody was used to detect the primary antibody.Immunocytochemistry/Immunofluorescence: Dihydrolipoamide Dehydrogenase/DLD Antibody [NBP1-31302] - HeLa cells were fixed in ice-cold MeOH for 5 min. Green: DLD protein stained by DLD antibody diluted at 1:500. Blue: Hoechst 33342 staining.

Rabbit Polyclonal
Species Human, Mouse, Rat
Applications WB, ICC/IF, IHC

     1 Review

Fumarase Antibody
NBP1-31336
Knockdown Validated: Fumarase Antibody [NBP1-31336] - Non-transfected (-) and transfected (+) 293T whole cell extracts (30 ug) were separated by 10% SDS-PAGE, and the membrane was blotted with Fumarate hydratase antibody. HRP-conjugated anti-rabbit IgG antibody was used to detect the primary antibody.Western Blot: Fumarase Antibody [NBP1-31336] - Representative western blots showing protein expression of fumarase and key enzymes in OXPHOS. VDAC was used as a loading control (n = 3). Image collected and cropped by CiteAb from the following publication (https://www.nature.com/articles/srep22788) licensed under a CC-BY license.

Rabbit Polyclonal
Species Human, Mouse, Rat
Applications WB, ICC/IF, IHC

     1 Review

1 Publication
Pyruvate Carboxylase Antibody - BSA Free
NBP1-49536
Immunocytochemistry/Immunofluorescence: Pyruvate Carboxylase Antibody [NBP1-49536] - HeLa cells were fixed in 4% paraformaldehyde for 10 min and permeabilized in 0.05% Triton X-100 in PBS for 5 minutes. The cells were incubated with anti- (NBP1-49536) at 10 ug/ml overnight at 4C and detected with an anti-rabbit Dylight 488 (Green) at a 1:500 dilution for 60 minutes. Nuclei were counterstained with DAPI (Blue). Cells were imaged using a 100X objective and digitally deconvolved.Western Blot: Pyruvate Carboxylase Antibody [NBP1-49536] - Analysis of Pyruvate Carboxylase in A. Human liver extracts and B. Mouse liver extracts

Rabbit Polyclonal
Species Human, Mouse, Rat
Applications WB, ICC/IF, IHC

17 Publications
PDHX Antibody
NBP1-84079
Western Blot: PDHX Antibody [NBP1-84079] - Analysis using Anti-PDHX antibody NBP1-84079 (A) shows similar pattern to independent antibody NBP1-84080 (B).Immunohistochemistry-Paraffin: PDHX Antibody [NBP1-84079] - Staining of human kidney.

Rabbit Polyclonal
Species Human
Applications WB, IHC, IHC-P

Pyruvate Dehydrogenase Phosphatase/PDP1/PPM2C Antibody
NBP1-87308
Immunohistochemistry-Paraffin: Pyruvate Dehydrogenase Phosphatase/PDP1/PPM2C Antibody [NBP1-87308] - Staining of human pancreas shows very weak granular cytoplasmic positivity in islets of Langerhans.Western Blot: Pyruvate Dehydrogenase Phosphatase/PDP1/PPM2C Antibody [NBP1-87308] - Analysis in human cell lines SK-MEL-30 and MCF-7. Corresponding RNA-seq data are presented for the same cell lines. Loading control: Anti-PFN1.

Rabbit Polyclonal
Species Human, Mouse, Rat
Applications WB, IHC, IHC-P

PNKD Antibody
NBP1-88347
Western Blot: PNKD Antibody [NBP1-88347] - Analysis in MCF-7 cells transfected with control siRNA, target specific siRNA probe #1 and #2, using Anti-PNKD antibody. Remaining relative intensity is presented. Loading control: Anti-GAPDH.Immunocytochemistry/Immunofluorescence: PNKD Antibody [NBP1-88347] - Immunofluorescent staining of human cell line U-2 OS shows localization to mitochondria.

Rabbit Polyclonal
Species Human, Mouse
Applications WB, ICC/IF, IHC

1 Publication
ALDH9A1 Antibody
NBP1-89150
Western Blot: ALDH9A1 Antibody [NBP1-89150] - Analysis in rat cell line NBT-II.Immunohistochemistry-Paraffin: ALDH9A1 Antibody [NBP1-89150] - Staining of human liver shows strong cytoplasmic positivity in hepatocytes.

Rabbit Polyclonal
Species Human, Rat
Applications WB, IHC, IHC-P

3 Publications
DBT Antibody
NBP1-89522
Immunohistochemistry-Paraffin: DBT Antibody [NBP1-89522] - Analysis in human kidney and pancreas tissues. Corresponding DBT RNA-seq data are presented for the same tissues.Immunohistochemistry-Paraffin: DBT Antibody [NBP1-89522] - Staining of human colon, liver, lymph node and pancreas using Anti-DBT antibody NBP1-89522 (A) shows similar protein distribution across tissues to independent antibody NBP1-85964 (B).

Rabbit Polyclonal
Species Human, Mouse, Rat
Applications WB, ICC/IF, IHC

Glut1 Antibody - BSA Free
NB110-39113
Western Blot: Glut1 Antibody [NB110-39113] - PrP-mediated increase in IC iron downregulates glucose transporters in the brain, neuroretina, and the liver: A similar evaluation of retinal lysates shows upregulation of Glut1 in PrP-/- relative to Tg40 PrP samples (lanes 1 & 3). Iron overloading downregulates Glut1 in Tg40 PrP samples relative to untreated controls, but has minimal effect on similarly treated PrP-/- samples (lanes 2 & 4). Image collected and cropped by CiteAb from the following publication (https://www.nature.com/articles/s41598-018-24786-1) licensed under a CC-BY license.Western Blot: Glut1 Antibody [NB110-39113] - Western blot of GLUT1 on mouse kidney membrane protein (lane A) and rat kidney membrane protein (lane B).

Rabbit Polyclonal
Species Human, Mouse, Rat
Applications WB, Simple Western, ChIP

     7 Reviews

62 Publications
MASA Antibody
NBP2-13961
Western Blot: MASA Antibody [NBP2-13961] - Analysis in control (vector only transfected HEK293T lysate) and ENOPH1 over-expression lysate (Co-expressed with a C-terminal myc-DDK tag (3.1 kDa) in mammalian HEK293T cells).Immunocytochemistry/Immunofluorescence: MASA Antibody [NBP2-13961] - Staining of human cell line MCF7 shows localization to nucleoplasm & nuclear bodies.

Rabbit Polyclonal
Species Human
Applications WB, ICC/IF, IHC

GM-CSF [Unconjugated]
7954-GM/CF
Measured in a cell proliferation assay using TF-1 human erythroleukemic cells. The ED<sub>50</sub> for this effect is 6-30 pg/mL.


Species Human
Applications BA

3 Publications
LAMC2 Antibody (CL2980)
NBP2-42388
Immunohistochemistry-Paraffin: LAMC2 Antibody (CL2980) [NBP2-42388] - Staining in human fallopian tube and liver tissues. Corresponding LAMC2 RNA-seq data are presented for the same tissues.Western Blot: LAMC2 Antibody (CL2980) [NBP2-42388] - Analysis in A-431 cells transfected with control siRNA, target specific siRNA probe #1 and #2, using Anti-LAMC2 antibody. Remaining relative intensity is presented. Loading control: Anti-GAPDH.

Mouse Monoclonal
Species Human
Applications WB, ICC/IF, IHC

4 Publications
Butyrylcholinesterase/BCHE Antibody [Unconjugated]
AF9024
Western blot shows lysates of mouse heart tissue and mouse liver tissue. PVDF membrane was probed with 1 µg/mL of Goat Anti-Mouse/Rat Butyrylcholinesterase/BCHE Polyclonal Antibody (Catalog # AF9024) followed by HRP-conjugated Anti-Goat IgG Secondary Antibody (Catalog # <a class=Butyrylcholinesterase/BCHE was detected in perfusion fixed frozen sections of mouse brain using Goat Anti-Mouse/Rat Butyrylcholinesterase/BCHE Antigen Affinity-purified Polyclonal Antibody (Catalog # AF9024) at 5 µg/mL overnight at 4 °C. Tissue was stained using the Anti-Goat HRP-DAB Cell & Tissue Staining Kit (brown; Catalog # <a class=

Goat Polyclonal
Species Mouse, Rat
Applications WB, IHC

2 Publications
Elastase Antibody (CELA3B/1257) - Azide and BSA Free
NBP2-54301
Western Blot: Elastase Antibody (CELA3B/1257) - Azide and BSA Free [NBP2-54301] - Western Blot Analysis of PANC-1 cell lysate using Elastase antibody (CELA3B/1257).Immunohistochemistry-Paraffin: Elastase Antibody (CELA3B/1257) - Azide and BSA Free [NBP2-54301] - Formalin-fixed, paraffin-embedded Mouse Pancreas stained with CELA3B Monoclonal Antibody (CELA3B/1257).

Mouse Monoclonal
Species Human, Mouse, Rat
Applications WB, ELISA, IHC

Pyruvate Dehydrogenase E1-alpha subunit [p Ser293] Antibody - BSA Free
NB110-93479
Western Blot: Pyruvate Dehydrogenase E1-alpha subunit [p Ser293] Antibody - BSA Free [NB110-93479] - Pyruvate Dehydrogenase E1-alpha subunit [p Ser293] Antibody [NB110-93479] - MEF were treated with 10 uM BA or DMSO (0.1%) for 16 h before they were subjected to a glycolysis stress test as described under Immunocytochemistry/Immunofluorescence: Pyruvate Dehydrogenase E1-alpha subunit [p Ser293] Antibody [NB110-93479] - Pyruvate Dehydrogenase E1-alpha subunit [p Ser293] antibody (1:250) was tested in HeLa cells with DyLight 488 (green). Nuclei and alpha-tubulin were counterstained with DAPI (blue) and DyLight 550 (red).

Rabbit Polyclonal
Species Human, Mouse, Rat
Applications WB, Flow, IB

     5 Reviews

57 Publications
Cytochrome c Antibody (7H8.2C12) - BSA Free
NB100-56503
Western Blot: Cytochrome c Antibody (7H8.2C12) [NB100-56503] - Metformin activates apoptotic cell death; Right panel: MCF-7 cells were kept in the presence or absence of metformin for 20 days and then processed to obtain mitochondrial or whole cellular extracts. BAX and CYCS expression levels were determined by Western blot as indicated under Material and Methods. Densitometric analysis of the gels was performed as indicated under Material and Methods. PHB and CDK4 were used as purity and loading controls. Image collected and cropped by CiteAb from the following publication (https://www.mdpi.com/2073-4409/8/1/49), licensed under a CC-BY license.Immunocytochemistry/Immunofluorescence: Cytochrome c Antibody (7H8.2C12) [NB100-56503] - p73 was detected in immersion fixed Hela human cell line using NB100-56503 at 25 ug/ml for 3 hours at room temperature. Cells were stained using the NorthernLights(TM) 557-conjugated Anti-Mouse IgG Secondary Antibody (red; Catalog # NL007) and counterstained with DAPI (blue). Staining was observed in the cytoplasm and mitochondria.

Mouse Monoclonal
Species Human, Mouse, Rat
Applications WB, Simple Western, Flow

61 Publications
ILT6/CD85e/LILRA3 Antibody (2E9)
H00011026-M01
Western Blot: ILT6/CD85e/LILRA3 Antibody (2E9) [H00011026-M01] - Analysis of LILRA3 expression in transfected 293T cell line by LILRA3 monoclonal antibody (M01), clone 2E9.Lane 1: LILRA3 transfected lysate(50.485 KDa).Lane 2: Non-transfected lysate.

Mouse Monoclonal
Species Human
Applications WB, ELISA

6 Publications
Phosducin Antibody - BSA Free
NBP2-94660
Western Blot: Phosducin Antibody [NBP2-94660] - Analysis of extracts of various cell lines, using Phosducin at 1:1000 dilution.Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) at 1:10000 dilution.Lysates/proteins: 25ug per lane.Blocking buffer: 3% nonfat dry milk in TBST.Detection: ECL Basic Kit .Exposure time: 10s.Immunohistochemistry-Paraffin: Phosducin Antibody [NBP2-94660] - Paraffin-embedded rat retina using Phosducin .

Rabbit Polyclonal
Species Human, Mouse, Rat
Applications WB, ELISA, IHC


Related Genes

Pyruvate Dehydrogenase Complex Deficiency Disease has been researched against:

Related Pathways

Pyruvate Dehydrogenase Complex Deficiency Disease has been linked to:

Related Diseases

Pyruvate Dehydrogenase Complex Deficiency Disease has been studied in relation to diseases such as:

Related PTMs

Pyruvate Dehydrogenase Complex Deficiency Disease has been studied in relation to posttranslational modifications (PTMs) including:

Alternate Names

Pyruvate Dehydrogenase Complex Deficiency Disease is also known as Ataxia With Lactic Acidosis, Deficiencies, Pyruvate Dehydrogenase, Deficiency Of Pyruvate Dehydrogenase, Deficiency, Pyruvate Decarboxylase, Deficiency, Pyruvate Dehydrogenase.