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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, IDURONATE SULFATASE, OSTEOPETROSIS, AGA, ARSA.

Top Research Reagents

We have 2019 products for the study of Pfaundler-hurler Syndrome that can be applied to Chromatin Immunoprecipitation (ChIP), Flow Cytometry, Immunocytochemistry/ Immunofluorescence, Immunohistochemistry, Western Blot from our catalog of antibodies and ELISA kits.

Arylsulfatase A/ARSA Antibody
NBP1-00154
Western Blot: Arylsulfatase A/ARSA Antibody [NBP1-00154] - Western blot analysis of Mouse testes lysate (A) + peptide (B) with antibody at 1 ug/mL and Rat testes lysate (C) + peptide (D) with antibody at 0.3 ug/mL, 35ug protein in RIPA buffer. Detected by chemiluminescence.Immunocytochemistry/Immunofluorescence: Arylsulfatase A/ARSA Antibody [NBP1-00154] - Immunofluorescence analysis of paraformaldehyde fixed HeLa cells, permeabilized with 0.15% Triton. Primary incubation 1hr (10 ug/mL) followed by Alexa Fluor 488 secondary antibody (2 ug/mL), showing Golgi apparatus staining. The nuclear stain is DAPI (blue). Negative control: Unimmunized goat IgG (10 ug/mL) followed by Alexa Fluor 488 secondary antibody (2 ug/mL).

Goat Polyclonal
Species Human, Mouse, Rat
Applications WB, Flow, ICC/IF

1 Publication
Recombinant Human ARSH GST (N-Term) Protein
H00347527-P01
12.5% SDS-PAGE Stained with Coomassie Blue.


Species Human
Applications WB, ELISA, PA

ELF3/ESE-1 Antibody
NBP1-30873
Western Blot: ELF3/ESE-1 Antibody [NBP1-30873] - Loss of Elf3 preserves the mesenchymal state. Immunoblot showing ELF3 and E-cadherin protein levels in response to Elf3 silencing by siRNAs. Image collected and cropped by CiteAb from the following publication (https://www.mdpi.com/2073-4409/8/8/858) licensed under a CC-BY license.Immunocytochemistry/Immunofluorescence: ELF3/ESE-1 Antibody [NBP1-30873] - Methanol-fixed HeLa, using antibody at 1:500 dilution.

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

3 Publications
N-Acetylgalactosamine-6-Sulfatase/GALNS Antibody
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

DHPS Antibody
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

2 Publications
Peripherin 2/PRPH2 Antibody
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

beta-Glucuronidase/GUSB Antibody
NBP1-87511
Immunohistochemistry-Paraffin: beta-Glucuronidase/GUSB Antibody [NBP1-87511] - Analysis in human liver and skeletal muscle tissues using NBP1-87511 antibody. Corresponding GUSB RNA-seq data are presented for the same tissues.Western Blot: beta-Glucuronidase/GUSB Antibody [NBP1-87511] - Analysis in control (vector only transfected HEK293T lysate) and GUSB over-expression lysate (Co-expressed with a C-terminal myc-DDK tag (3.1 kDa) in mammalian HEK293T cells).

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

AGA Antibody
NBP1-88866
Immunohistochemistry-Paraffin: AGA Antibody [NBP1-88866] - Staining in human epididymis and skeletal muscle tissues using anti-AGA antibody. Corresponding AGA RNA-seq data are presented for the same tissues.Immunohistochemistry-Paraffin: AGA Antibody [NBP1-88866] - Staining of human epididymis shows high expression.

Rabbit Polyclonal
Species Human
Applications IHC, IHC-P

1 Publication
Elastin Antibody (BA-4)
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] - Formalin fixed paraffin embedded human renal cell carcinoma stained with Elastin antibody (NB100-2076)

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

     2 Reviews

11 Publications
NIK/MAP3K14 Antibody - BSA Free
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

1 Publication
CD34 Antibody [Unconjugated]
AF4117
CD34 was detected in perfusion fixed frozen sections of rat liver using 15 µg/mL Rat CD34 Antigen Affinity-purified Polyclonal Antibody (Catalog # AF4117) overnight at 4 °C. Tissue was stained with the Anti-Goat HRP-DAB Cell & Tissue Staining Kit (brown; Catalog # <a class=

Goat Polyclonal
Species Rat
Applications WB, IHC

     3 Reviews

37 Publications
Arylsulfatase B/ARSB Antibody (708401)
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=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=

Mouse Monoclonal
Species Human
Applications WB, ICC

     1 Review

4 Publications
Cathepsin A/Lysosomal Carboxypeptidase A Antibody
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=

Goat Polyclonal
Species Human
Applications WB, IHC

     1 Review

6 Publications
alpha-L-Iduronidase/IDUA Antibody [Unconjugated]
AF4119
Western blot shows lysates of human kidney tissue and IMR-90 human lung fibroblast cell line. PVDF membrane was probed with 1 µg/mL of Sheep Anti-Human a-L-Iduronidase/IDUA Antigen Affinity-purified Polyclonal Antibody (Catalog # AF4119) followed by HRP-conjugated Anti-Sheep IgG Secondary Antibody (Catalog # <a class=a-L-Iduronidase/IDUA was detected in immersion fixed HepG2 human hepatocellular carcinoma cell line using Sheep Anti-Human a-L-Iduronidase/IDUA Antigen Affinity-purified Polyclonal Antibody (Catalog # AF4119) at 15 µg/mL for 3 hours at room temperature. Cells were stained using the NorthernLights™ 557-conjugated Anti-Sheep IgG Secondary Antibody (red; Catalog # <a class=

Sheep Polyclonal
Species Human
Applications WB, IP, ICC

4 Publications
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
beta-Galactosidase-1/GLB1 Antibody (OTI1C9)
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

6 Publications

Related Genes

Pfaundler-hurler Syndrome has been researched against:

Related Pathways

Pfaundler-hurler Syndrome has been linked to:

Related Diseases

Pfaundler-hurler Syndrome has been studied in relation to diseases such as:

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.