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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 IL-1BETA, ALDH9A1, BCHE, BCKDHA, BCKDHB.

Top Research Reagents

We have 4272 products for the study of Maple Syrup Urine Disease that can be applied to Chromatin Immunoprecipitation (ChIP), Flow Cytometry, Immunocytochemistry/ Immunofluorescence, Immunohistochemistry, Western Blot from our catalog of antibodies and ELISA kits.

IL-6 [HRP]
D6050B
N/A IL-6 [HRP]N/A IL-6 [HRP]


Species Human

776 Publications
BCKDHA Antibody
NBP1-79616
Western Blot: BCKDHA Antibody [NBP1-79616] - MCF-7 whole cell lysates, concentration 0.2-1 ug/ml.Immunohistochemistry-Paraffin: 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, IHC, IHC-P

3 Publications
BCKDHB Antibody
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

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

Dihydrolipoamide Dehydrogenase/DLD Antibody (OTI6D5)
NBP2-01109
Western Blot: Lipoamide Dehydrogenase Antibody (6D5) [NBP2-01109] - HEK293T cells were transfected with the pCMV6-ENTRY control (Left lane) or pCMV6-ENTRY Lipoamide Dehydrogenase (Right lane) cDNA for 48 hrs and lysed. Equivalent amounts of cell lysates (5 ug per lane) were separated by SDS-PAGE and immunoblotted with anti-Lipoamide Dehydrogenase.Immunocytochemistry/Immunofluorescence: Lipoamide Dehydrogenase Antibody (6D5) [NBP2-01109] Staining of COS7 cells transiently transfected by pCMV6-ENTRY Lipoamide Dehydrogenase.

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

1 Publication
NLRP3/NALP3 Antibody - BSA Free
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 (https://journal.frontiersin.org/article/10.3389/fimmu.2016.00532/full), licensed under a CC-BY license.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, Flow, ICC/IF

     4 Reviews

232 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

ERK1 Antibody (1E5) - BSA Free
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

1 Publication
TNF-alpha [Unconjugated]
210-TA
Recombinant Human TNF-alpha (Catalog # 210-TA) has a molecular weight (MW) of 53.1 kDa as analyzed by SEC-MALS, suggesting that this protein is a homotrimer.  MW may differ from predicted MW due to post-translational modifications (PTMs) present (i.e. Glycosylation).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
Applications BA

     3 Reviews

815 Publications
IL-1 beta/IL-1F2 [Unconjugated]
201-LB
Recombinant Human IL-1beta Protein (Catalog # 201-LB) has a molecular weight (MW) of 17.0 kDa as analyzed by SEC-MALS, suggesting that this protein is a monomer.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
Applications BA

594 Publications
CXCL8/IL-8 [Biotin]
DY208
N/A CXCL8/IL-8 [Biotin]


Species Human
Applications ELISA

464 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
Phenylalanine Hydroxylase Antibody
NBP2-48615
Immunohistochemistry-Paraffin: Phenylalanine Hydroxylase Antibody [NBP2-48615] - Analysis in human liver and pancreas tissues. Corresponding Phenylalanine Hydroxylase RNA-seq data are presented for the same tissues.Immunohistochemistry-Paraffin: Phenylalanine Hydroxylase Antibody [NBP2-48615] - Staining of human cerebral cortex, kidney, liver and pancreas using Anti-Phenylalanine Hydroxylase antibody NBP2-48615 (A) shows similar protein distribution across tissues to independent antibody NBP1-80917 (B).

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

1 Publication
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

BRCAA1 Antibody
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, IHC, IHC-P

     1 Review

1 Publication
Biotinidase/BTD Antibody
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

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
TLR4 Antibody (76B357.1) - BSA Free
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, DB, ELISA

     5 Reviews

140 Publications

Related Genes

Maple Syrup Urine Disease has been researched against:

Related Pathways

Maple Syrup Urine Disease has been linked to:

Related Diseases

Maple Syrup Urine Disease has been studied in relation to diseases such as:

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.