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Maple Syrup Urine Disease: Disease Bioinformatics

Research of Maple Syrup Urine Disease has been linked to Phenylketonurias, Inborn Errors Of Metabolism, Metabolic Diseases, Gout, Inflammation. The study of Maple Syrup Urine Disease has been mentioned in research publications which can be found using our bioinformatics tool below. Researched pathways related to Maple Syrup Urine Disease include Pathogenesis, Inflammatory Response, Phagocytosis, Excretion, Transport. These pathways complement our catalog of research reagents for the study of Maple Syrup Urine Disease including antibodies and ELISA kits against BCKDHA, DBT, TNF, ARID4B, PAH.

Maple Syrup Urine Disease Bioinformatics Tool

Laverne is a handy bioinformatics tool to help facilitate scientific exploration of related genes, diseases and pathways based on co-citations. Explore more on Maple Syrup Urine Disease below! For more information on how to use Laverne, please read the How to Guide.
Vizit™, under license from BioVista Inc.

Top Research Reagents

We have 2167 products for the study of Maple Syrup Urine Disease that can be applied to Chromatin Immunoprecipitation, Western Blot, Flow Cytometry, Immunohistochemistry, Immunocytochemistry/Immunofluorescence from our catalog of antibodies and ELISA kits.

NBP1-79616
Western Blot: BCKDHA Antibody [NBP1-79616] - MCF-7 whole cell lysates, concentration 0.2-1 ug/ml.Immunocytochemistry/Immunofluorescence: BCKDHA Antibody [NBP1-79616] - Formalin Fixed Paraffin Embedded Tissue: Human Lung Tissue  Observed Staining: Cytoplasmic in alveolar type I & II cells

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

3 Publications
NBP1-89522
Western Blot: DBT Antibody [NBP1-89522] - Lane 1: NIH-3T3 cell lysate (Mouse embryonic fibroblast cells). Lane 2: NBT-II cell lysate (Rat Wistar bladder tumor cells).Immunocytochemistry/Immunofluorescence: DBT Antibody [NBP1-89522] - Staining of human cell line A-431 shows localization to mitochondria. Antibody staining is shown in green.

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

210-TA
1 µg/lane of Recombinant Human TNF-alpha  was resolved by SDS-PAGE with silver staining, under reducing (R) conditions, showing a band at 17 kDa.Recombinant Human TNF-alpha  (Catalog # 210‑TA) induces cytotoxicity in the L-929 mouse fibroblast cell line in the presence of the metabolic inhibitor actinomycin D. The ED<sub>50</sub> for this effect is 25‑100 pg/mL.


Species Human

     32 Reviews

647 Publications
NBP1-26618
Western Blot: BRCAA1 Antibody [NBP1-26618] - Whole cell lysate from HeLa cells. ARID4B was also immunoprecipitated by rabbit anti-ARID4B antibody NBP1-26619.Immunohistochemistry: BRCAA1 Antibody [NBP1-26618] - Sample: FFPE section of human ovarian carcinoma. Antibody: Affinity purified rabbit anti-ARID4B used at a dilution of 1:1,000 ( 1ug/ml). Detection: DAB staining using Immunohistochemistry Accessory Kit.

Rabbit Polyclonal
Species Human
Applications WB, ChIP, IHC

     1 Review

1 Publication
NBP1-52084
Western Blot: Phenylalanine Hydroxylase Antibody [NBP1-52084] - (0.1ug/ml) staining of Mouse (A) and Rat (B) Liver lysate (35ug protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.Immunohistochemistry-Paraffin: Phenylalanine Hydroxylase Antibody [NBP1-52084] - Staining of paraffin embedded Human Kidney. Steamed antigen retrieval with citrate buffer pH 6, AP-staining.

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

1 Publication
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

NBP2-12446
Western Blot: NLRP3/NALP3 Antibody [NBP2-12446] - C-AR inhibited NLRP3 inflammasome activation in synovial tissue. NLRP3 protein expression in succinate-stimulated synovial fibroblasts. The results were derived from four independent experiments for immunohistochemistry staining and Western blot and expressed as the mean +/- SD. *p < 0.05 vs. the model; #p < 0.05 vs. the indicated treatment. Image collected and cropped by CiteAb from the following publication (http://journal.frontiersin.org/article/10.3389/fimmu.2016.00532/full), licensed under a CC-BY licence.Immunohistochemistry-Paraffin: NLRP3/NALP3 Antibody [NBP2-12446] - Analysis of human esophagus using NLRP3/NALP3 antibody at 1:50 on a Bond Rx autostainer (Leica Biosystems). The assay involved 20 minutes of heat induced antigen retrieval (HIER) using 10mM sodium citrate buffer (pH 6.0) and endogenous peroxidase quenching with peroxide block. The sections were incubated with primary antibody for 30 minutes and Bond Polymer Refine Detection (Leica Biosystems) with DAB was used for signal development followed by counterstaining with hematoxylin. Whole slide scanning and capturing of representative images was performed using Aperio AT2 (Leica Biosystems). Cytoplasmic staining in the squamous epithelium was observed. Staining was performed by Histowiz.

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

     4 Reviews

88 Publications
D6050
 IL-6 [HRP] IL-6 [HRP]


Species Human

     25 Reviews

465 Publications
201-LB
1 μg/lane of Recombinant Human IL-1 beta /IL-1F2 was resolved with SDS-PAGE under reducing (R) conditions and visualized by silver staining, showing a single band at 17 kDa.Recombinant Human IL-1 beta /IL-1F2 (Catalog # 201-LB) stimulates cell proliferation of the D10.G4.1 mouse helper T cell line. The ED<SUB>50</SUB> for this effect is <12 pg/mL.


Species Human

     18 Reviews

403 Publications
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 <br>(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

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

NBP2-53246
Immunohistochemistry-Paraffin: Elastase Antibody (CELA3B/1257) [NBP2-53246] - Formalin-fixed, paraffin-embedded Rat Pancreas stained with CELA3B Monoclonal Antibody (CELA3B/1257).Immunohistochemistry-Paraffin: Elastase Antibody (CELA3B/1257) [NBP2-53246] - Formalin-fixed, paraffin-embedded Human Pancreas stained with CELA3B Monoclonal Antibody (CELA3B/1257)

Mouse Monoclonal
Species Human, Mouse, Rat
Applications ELISA, Flow, ICC/IF

D8000C
 CXCL8/IL-8 [HRP] CXCL8/IL-8 [HRP]


Species Human

     8 Reviews

314 Publications
NBP2-97093
Western Blot: Biotinidase/BTD Antibody [NBP2-97093] - Anti-Biotinidase/BTD rabbit polyclonal antibody at 1:500 dilution. Lane A: Jurkat Whole Cell Lysate. Lysates/proteins at 30 ug per lane. Secondary Goat Anti-Rabbit IgG H&L (Dylight 800) at 1/10000 dilution. Developed using the Odyssey technique. Performed under reducing conditions. Predicted band size: 61 kDa. Observed band size: 48 kDaImmunohistochemistry-Paraffin: Biotinidase/BTD Antibody [NBP2-97093] - Immunochemical staining of human Biotinidase/BTD in human liver with rabbit polyclonal antibody (1:5000, formalin-fixed paraffin embedded sections).

Rabbit Polyclonal
Species Human
Applications WB, ELISA, IHC

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
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

5 Publications
NBP2-22203
Western Blot: ERK1 Antibody (1E5) [NBP2-22203] - Western blot analysis of whole cell lysates from (1) MCF7 (2) NIH3T3 cell lines using ERK1 antibody (clone 1E5) at 1:1000 dilution. The signal was developed using HRP labeled goat-anti Mouse secondary antibody with ECL based detection. Immunocytochemistry/Immunofluorescence: ERK1 Antibody (1E5) [NBP2-22203] - Analysis of NIH/3T3 cells using ERK1 mouse mAb (green). Blue: DRAQ5 fluorescent DNA dye. Red: Actin filaments have been labeled with Alexa Fluor-555 phalloidin.

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

NB100-56566
Dual RNAscope ISH-IHC: TLR4 Antibody (76B357.1) [NB100-56566] - FFPE tissue sections of human tonsil were probed for TLR4 mRNA (ACD RNAScope Probe, ACD catalog # 311281; Fast Red chromogen, ACD catalog # 322750). Adjacent tissue section was processed for immunohistochemistry using Mouse Monoclonal (Novus Biologicals catalog # NB100-56566) at 5ug/mL with 1 hour incubation at room temperature followed by incubation with anti-mouse IgG VisUCyte HRP Polymer Antibody (Catalog # VC001) and DAB chromogen (yellow-brown). Tissue was counterstained with hematoxylin (blue). Specific staining was localized to lymphocytes.Flow Cytometry: TLR4 Antibody (76B357.1) [NB100-56566] - Analysis using the Alexa Fluor (R) 647 conjugate of NBP2-27149. TLR4 expression on monocytes from human peripheral blood. PBMC were stained in a 2 color flow test, with CD14 PE version of this antibody and 1 ug of either isotype control (left) or TLR4-Alexa Fluor 647 (right). PPI negative, CD14+ cells were gated for analysis.

Mouse Monoclonal
Species Human, Mouse, Rat
Applications WB, ChIP, DB

     5 Reviews

101 Publications
NBP1-86326
Western Blot: BCKDHB Antibody [NBP1-86326] - Analysis in control (vector only transfected HEK293T lysate) and BCKDHB over-expression lysate (Co-expressed with a C-terminal myc-DDK tag (3.1 kDa) in mammalian HEK293T cells).Immunohistochemistry-Paraffin: BCKDHB Antibody [NBP1-86326] - Staining of human fallopian tube shows strong granular cytoplasmic positivity in glandular cells.

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


Related Genes

Maple Syrup Urine Disease has been researched against:

Related PTMs

Maple Syrup Urine Disease has been studied in relation to posttranslational modifications (PTMs) including:

Alternate Names

Maple Syrup Urine Disease is also known as maple syrup urine disease, bckd deficiency, branched chain ketoaciduria, maple syrup disease, keto acid decarboxylase deficiency, ketoacidaemia, ketoacidemia, ketonemia.