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Leigh Disease: Disease Bioinformatics

Research of Leigh Disease has been linked to Mitochondrial Diseases, Encephalopathies, Cytochrome-c Oxidase Deficiency, Acidosis, Acidosis, Lactic. The study of Leigh Disease has been mentioned in research publications which can be found using our bioinformatics tool below. Researched pathways related to Leigh Disease include Oxidative Phosphorylation, Pathogenesis, Translation, Transport, Localization. These pathways complement our catalog of research reagents for the study of Leigh Disease including antibodies and ELISA kits against MT-ATP6, ATP5E, COX8A, CPOX, CSF2.

Top Research Reagents

We have 1097 products for the study of Leigh Disease that can be applied to Flow Cytometry, Immunocytochemistry/ Immunofluorescence, Immunohistochemistry, Western Blot from our catalog of antibodies and ELISA kits.

Recombinant Human COX8A GST (N-Term) Protein
H00001351-P01
SDS-Page: COX8A Recombinant Protein [H00001351-P01] - 12.5% SDS-PAGE Stained with Coomassie Blue.


Species Human
Applications WB, ELISA, PA

Surf1 Antibody
NBP3-41876
Immunohistochemistry-Paraffin: Surf1 Antibody [NBP3-41876] - Used in DAB staining on fromalin fixed paraffin- embedded kidney tissue

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

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

COX5A Antibody
NBP1-32550
Western Blot: COX5A Antibody [NBP1-32550] - Various whole cell extracts (30 ug) were separated by 15% SDS-PAGE, and the membrane was blotted with COX5A antibody [N1C3] diluted at 1:5000. The HRP-conjugated anti-rabbit IgG antibody (NBP2-19301) was used to detect the primary antibody, and the signal was developed with Trident ECL plus-Enhanced.Immunocytochemistry/Immunofluorescence: COX5A Antibody [NBP1-32550] - A431.

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

3 Publications
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
NDUFS7 Antibody
NBP1-49846
Western Blot: NDUFS7 Antibody [NBP1-49846] - (35ug protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.Immunohistochemistry-Paraffin: NDUFS7 Antibody [NBP1-49846] - Staining of paraffin embedded Human Liver at dilution of 5ug/ml. Steamed antigen retrieval with citrate buffer pH 6, AP-staining.

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

1 Publication
DNA Polymerase gamma Antibody
NBP1-52300
Western Blot: DNA Polymerase gamma Antibody [NBP1-52300] - Analysis of anti-POLG antibody with (30 ug of whole cell lysate). A: 293T. 5% SDS PAGE. POLG antibody diluted at 1:5000.Immunocytochemistry/Immunofluorescence: DNA Polymerase gamma Antibody [NBP1-52300] - Analysis of anti-POLG antibody with methanol-fixed HeLa cell at 1:200 dilution.

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

5 Publications
Neuronal Pentraxin 2 Antibody - BSA Free
NBP1-76899
Western Blot: Neuronal Pentraxin 2 Antibody [NBP1-76899] - Independent Antibody Validation (IAV) via Protein Expression Profile in Cell Lines. Loading: 15 ug of lysates per lane. Antibodies: Neuronal Pentraxin 2 NBP1-76899 (2 ug/mL), Neuronal Pentraxin 2 (4 ug/mL), and beta-actin (1 ug/mL), 1h incubation at RT in 5% NFDM/TBST. Secondary: Goat anti-rabbit IgG HRP conjugate at 1:10000 dilution.Western Blot: Neuronal Pentraxin 2 Antibody [NBP1-76899] - Mouse brain tissue lysate with NPTX2 antibody at (A) 0.5 and (B) 1 ug/mL.

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

SCO2 Antibody - BSA Free
NBP1-77274
Western Blot: SCO2 Antibody [NBP1-77274] - Analysis in HL60 cell lysate lysate with antibody at (A) 1 and (B) 2 ug/mL.Immunohistochemistry-Paraffin: SCO2 Antibody [NBP1-77274] - Human liver tissue with SCO2 antibody at 2.5 ug/ml.

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

LIAS Antibody
NBP1-82845
Immunocytochemistry/Immunofluorescence: LIAS Antibody [NBP1-82845] - Immunofluorescent staining of human cell line U-2 OS shows localization to nucleoplasm & mitochondria. Antibody staining is shown in green.Immunohistochemistry: LIAS Antibody [NBP1-82845] - Staining of mouse lateral ventricle shows positivity in a subset of cells in choroid plexus and lateral ventricle wall.

Rabbit Polyclonal
Species Human, Mouse
Applications ICC/IF, IHC, IHC-P

LRPPRC Antibody
NBP1-83349
Immunohistochemistry-Paraffin: LRPPRC Antibody [NBP1-83349] - Staining of human cerebellum, colon, kidney and liver using Anti-LRPPRC antibody NBP1-83349 (A) shows similar protein distribution across tissues to independent antibody NBP2-38300 (B).Western Blot: LRPPRC Antibody [NBP1-83349] - Analysis in Hep-G2 cells transfected with control siRNA, target specific siRNA probe #1 and #2, using Anti-LRPPRC antibody. Remaining relative intensity is presented. Loading control: Anti-GAPDH.

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

1 Publication
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

Ndufs4 Antibody
NBP1-89026
Western Blot: Ndufs4 Antibody [NBP1-89026] - Analysis in HEK293 cells transfected with control siRNA, target specific siRNA probe #1 and #2. Remaining relative intensity is presented. Loading control: Anti-GAPDH.Immunocytochemistry/Immunofluorescence: Ndufs4 Antibody [NBP1-89026] - Immunofluorescent staining of human cell line U-2 OS shows localization to mitochondria.

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

1 Publication
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
CPOX Antibody (36B10) - BSA Free
NBP2-59438
Western Blot: CPOX Antibody (36B10) [NBP2-59438] - Lane 1: Recombinant protein CPOX, Lane 2: 293T cell lysates, Lane 3: HepG2 cell lysates, Lane 4: A549 cell lysates, Lane 5: Jurkat cell lysates, Lane 6: K562 cell lysates, Lane 7: LnCaP cell lysatesImmunocytochemistry/Immunofluorescence: CPOX Antibody (36B10) [NBP2-59438] - Analysis of CPOX in Hep3B cells line, stained with DAPI (Blue) for nucleus staining and monoclonal anti-human CPOX antibody (1:100) with goat anti-mouse IgG-Alexa fluor 488 conjugate (Green).

mouse Monoclonal
Species Human
Applications WB, ELISA, ICC/IF

1 Publication
Recombinant Human ATP5E GST (N-Term) Protein
H00000514-P01
SDS-Page: Recombinant Human ATP5E Protein [H00000514-P01] - 12.5% SDS-PAGE Stained with Coomassie Blue.


Species Human
Applications WB, ELISA, PA

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
MT-ND5 Antibody - BSA Free
NBP3-05606
Western Blot: MT-ND5 Antibody [NBP3-05606] - Western blot analysis of extracts of various cell lines, using MT-ND5 antibody (NBP3-05606) 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: 5s.Immunohistochemistry-Paraffin: MT-ND5 Antibody [NBP3-05606] - Immunohistochemistry of paraffin-embedded human prostate cancer using MT-ND5 antibody (NBP3-05606) at dilution of 1:100 (40x lens).

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


Related Genes

Leigh Disease has been researched against:

Related Pathways

Leigh Disease has been linked to:

Related Diseases

Leigh Disease has been studied in relation to diseases such as:

Related PTMs

Leigh Disease has been studied in relation to posttranslational modifications (PTMs) including:

Alternate Names

Leigh Disease is also known as leigh disease, leigh syndrome, leigh's disease, mri scans, necrotizing encephalopathy infantile subacute of leigh, encephalopathy, subacute necrotizing, infantile, encephalopathy, subacute necrotizing, juvenile, infantile subacute necrotizing encephalopathy, juvenile subacute necrotizing encephalopathy, infantile necrotizing encephalomyelopathy, subacute necrotizing encephalomyelopathy, subacute necrotizing encephalopathy, leigh's necrotizing encephalopathy, leigh's disease (disorder), sne, ls.