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Pfaundler-hurler Syndrome: Disease Bioinformatics

Research of Pfaundler-hurler Syndrome has been linked to Mucopolysaccharidosis I, Mucopolysaccharidoses, Dysostoses, Mucopolysaccharidosis Ii, Lysosomal Storage Diseases. The study of Pfaundler-hurler Syndrome has been mentioned in research publications which can be found using our bioinformatics tool below. Researched pathways related to Pfaundler-hurler Syndrome include Excretion, Transport, Secretion, Localization, Pathogenesis. These pathways complement our catalog of research reagents for the study of Pfaundler-hurler Syndrome including antibodies and ELISA kits against ALPHA-L-IDURONIDASE, IDUA, GLB1, ARSB, MAP3K14.

Pfaundler-hurler Syndrome 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 Pfaundler-hurler Syndrome below! For more information on how to use Laverne, please read the How to Guide.
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Top Research Reagents

We have 1133 products for the study of Pfaundler-hurler Syndrome that can be applied to Western Blot, Immunocytochemistry/Immunofluorescence, Flow Cytometry, Immunohistochemistry, Chromatin Immunoprecipitation (ChIP) from our catalog of antibodies and ELISA kits.

MAB4119
    Western  blot shows lysates of human kidney tissue and IMR‑90 human lung  fibroblast cell line. PVDF membrane was probed with 2 µg/mL of Mouse  Anti-Human  alpha ‑L‑Iduronidase/IDUA Monoclonal Antibody  (Catalog # MAB4119) followed by HRP-conjugated Anti-Mouse IgG Secondary  Antibody (Catalog # <a class=

Mouse Monoclonal
Species Human
Applications WB

     2 Reviews

NBP2-45731
Western Blot: beta-Galactosidase-1/GLB1 Antibody (OTI1C9) [NBP2-45731] - Analysis of extracts (35ug) from 9 different cell lines by using anti-GLB1 monoclonal antibody (HepG2: human; HeLa: human; SVT2: mouse; A549: human; COS7: monkey; Jurkat: human; MDCK: canine; PC12: rat; MCF7: human).Immunohistochemistry: beta-Galactosidase-1/GLB1 Antibody (1C9) [NBP2-45731] - Analysis of Adenocarcinoma of Human ovary tissue. (Heat-induced epitope retrieval by 10mM citric buffer, pH6.0, 120C for 3min)

Mouse Monoclonal
Species Human, Canine, Monkey
Applications WB, IHC, IHC-P

2 Publications
MAB4415
Western blot shows lysates of HepG2 human hepatocellular carcinoma cell line, Huh‑7 human hepatoma cell line, and human skeletal muscle tissue. PVDF membrane was probed with 1 µg/mL of Mouse Anti-Human Arylsulfatase B/ARSB Monoclonal Antibody (Catalog # MAB4415) followed by HRP-conjugated Anti-Mouse IgG Secondary Antibody (Catalog # <a class=NoLineLink href=Arylsulfatase B/ARSB was detected in immersion fixed HepG2 human hepatocellular carcinoma cell line using Mouse Anti-Human Arylsulfatase B/ARSB Monoclonal Antibody (Catalog # MAB4415) at 10 µg/mL for 3 hours at room temperature. Cells were stained using the NorthernLights™ 557-conjugated Anti-Mouse IgG Secondary Antibody (red; Catalog # <A class=NoLineLink href=

Mouse Monoclonal
Species Human
Applications WB, ICC

     1 Review

2 Publications
NBP2-23603
Western Blot: NIK/MAP3K14 Antibody [NBP2-23603] - Detection of MAP3K14 in U2OS cell lysate.Immunocytochemistry/Immunofluorescence: NIK/MAP3K14 Antibody [NBP2-23603] - MAP3K14 antibody was tested in U2OS cells with DyLight 488 (green). Nuclei and alpha-tubulin were counterstained with DAPI (blue) and Dylight 550 (red).

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

NBP1-30873
Western Blot: ELF3/ESE-1 Antibody [NBP1-30873] -  Sample (30 ug of whole cell lysate) A: A431 10% SDS PAGE; antibody diluted at 1:1000.Immunocytochemistry/Immunofluorescence: ELF3/ESE-1 Antibody [NBP1-30873] - Methanol-fixed HeLa, using antibody at 1:500 dilution.

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

2 Publications
NBP1-86135
Western Blot: Arylsulfatase A/ARSA Antibody [NBP1-86135] - Analysis in human cell line Jurkat.Immunohistochemistry-Paraffin: Arylsulfatase A/ARSA Antibody [NBP1-86135] - Staining of human prostate shows strong granular cytoplasmic positivity in glandular cells.

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

AF6144
Western blot shows lysates of HT‑29 human colon adenocarcinoma cell line. PVDF Membrane was probed with 1 µg/mL of Human  beta ‑Glucuronidase/GUSB Antigen Affinity-purified Polyclonal Antibody (Catalog # AF6144) followed by HRP-conjugated Anti-Sheep IgG Secondary Antibody (Catalog # <a class=Simple Western lane view shows lysates of HT‑29 human colon adenocarcinoma cell line, loaded at 0.2 mg/mL. A specific band was detected for  beta ‑Glucuronidase/GUSB at approximately 92 kDa (as indicated) using 10 µg/mL of Sheep Anti-Human  beta ‑Glucuronidase/GUSB Antigen Affinity-purified Polyclonal Antibody (Catalog # AF6144) followed by 1:50 dilution of HRP-conjugated Anti-Sheep IgG Secondary Antibody (Catalog # <a class=

Sheep Polyclonal
Species Human
Applications WB, Simple Western

1 Publication
NB600-1071
Immunocytochemistry/Immunofluorescence: CD34 Antibody (MEC 14.7) [NB600-1071] - CD34 antibody was tested in WEHI-3 cells with DyLight 488 (green). Nuclei and alpha-tubulin were counterstained with DAPI (blue) and DyLight 550 (red).Immunohistochemistry-Paraffin: CD34 Antibody (MEC 14.7) [NB600-1071] - Analysis of a FFPE tissue section of mouse small intestine using rat anti-mouse CD34 (clone MEC 14.7) at 1:100 dilution. The signal was developed using HRP-conjugated anti-rat secondary with DAB reagent which followed counterstaining of nuclei using hematoxylin. This antibody specifically labelled the endothelial cells in blood vessels located primarily in the sub-mucosa, and of that of the mucosa muscularis and the mucosal lacteal.

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

19 Publications
H00347527-P01
12.5% SDS-PAGE Stained with Coomassie Blue.


Species Human

NB100-2076
Western Blot: Elastin Antibody (BA-4) [NB100-2076] - Analysis of tropoelastin using the mAb BA-4 elastin antibody (NB100-2076). A: Western blot of tropoelastin purified from bovine ligamentum nuchae tissue minces. B: Autoradiogram of a gradient SDS-polyacrylamide gel showing [3H] leucine-labeled tropoelasti.Immunohistochemistry-Paraffin: Elastin Antibody (BA-4) [NB100-2076] - Presence of ECM proteins collagen, elastin, fibronectin, vitronectin and GAGs following decellularization. Porcine vena cavas were decellularized and analyzed for ECM proteins. Cross-section of paraffin embedded samples were immunostained for collagen type I/III, elastin, fibronectin and vitronectin. Images were taken at identical microscopy settings. Scale bar is 100 um. Image collected and cropped by CiteAb from the following publication (http://dx.plos.org/10.1371/journal.pone.0220743), licensed under a CC-BY licence.

Mouse Monoclonal
Species Human, Porcine, Canine
Applications WB, IHC, IHC-Fr

     2 Reviews

4 Publications
AF1049
Cathepsin A/Lysosomal Carboxypeptidase A was detected in immersion fixed paraffin-embedded sections of human prostate using Goat Anti-Human Cathepsin A/Lysosomal Carboxypeptidase A Antigen Affinity-purified Polyclonal Antibody (Catalog # AF1049) at 3 µg/mL overnight at 4 °C. Before incubation with the primary antibody, tissue was subjected to heat-induced epitope retrieval using Antigen Retrieval Reagent-Basic (Catalog # <A class=NoLineLink href=

Goat Polyclonal
Species Human
Applications WB, IHC

     1 Review

2 Publications
7954-GM/CF


Species Human

1 Publication
NBP1-86687
Western Blot: Peripherin 2/PRPH2 Antibody [NBP1-86687] - Analysis in control (vector only transfected HEK293T lysate) and PRPH2 over-expression lysate (Co-expressed with a C-terminal myc-DDK tag (3.1 kDa) in mammalian HEK293T cells).Immunohistochemistry-Paraffin: Peripherin 2/PRPH2 Antibody [NBP1-86687] - Staining of human liver shows no positivity in hepatocytes as expected.

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

NBP2-52631
SDS-Page: Recombinant Human AGA Protein [NBP2-52631]


Species Human

1 Publication
NBP1-32899
Western Blot: N-Acetylgalactosamine-6-Sulfatase/GALNS Antibody [NBP1-32899] - A. 30 ug PC-12 whole cell lysate/extract  B. 30 ug Rat2 whole cell lysate/extract7.5% SDS-PAGE GALNS antibody dilution: 1:500.  The HRP-conjugated anti-rabbit IgG antibody (NBP2-19301) was used to detect the primary antibody.Immunocytochemistry/Immunofluorescence: N-Acetylgalactosamine-6-Sulfatase/GALNS Antibody [NBP1-32899] - Paraformaldehyde-fixed HeLa, using GALNS antibody (Green) at 1:500 dilution. Alpha-tubulin filaments were labeled Red.

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

     2 Reviews

NBP1-82648
Western Blot: DHPS Antibody [NBP1-82648] - Analysis in control (vector only transfected HEK293T lysate) and DHPS over-expression lysate (Co-expressed with a C-terminal myc-DDK tag (3.1 kDa) in mammalian HEK293T cells).Immunocytochemistry/Immunofluorescence: DHPS Antibody [NBP1-82648] - Staining of human cell line U-2 OS shows localization to nucleoplasm, plasma membrane & cytosol. Antibody staining is shown in green.

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

1 Publication
NBP2-42388
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.Immunocytochemistry/Immunofluorescence: LAMC2 Antibody (CL2980) [NBP2-42388] - Staining of A-431 cells using the Anti-LAMC2 monoclonal antibody, showing specific staining in the cytosol in green. Microtubule- and nuclear probes are visualized in red and blue, respectively (where available).

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

2 Publications

Related Genes

Pfaundler-hurler Syndrome has been researched against:

Related PTMs

Pfaundler-hurler Syndrome has been studied in relation to posttranslational modifications (PTMs) including:

Alternate Names

Pfaundler-hurler Syndrome is also known as Disease, Hurler, Disease, Hurler's, Dysostosis Multiplex, Dysostosis Multiplex Syndrome, Gargoylism.