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Lysosomal Storage Diseases: Disease Bioinformatics

Research of Lysosomal Storage Diseases has been linked to Storage Disease, Mucopolysaccharidoses, Gaucher Disease, Nervousness, Fabry Disease. The study of Lysosomal Storage Diseases has been mentioned in research publications which can be found using our bioinformatics tool below. Researched pathways related to Lysosomal Storage Diseases include Transport, Pathogenesis, Excretion, Endocytosis, Autophagy. These pathways complement our catalog of research reagents for the study of Lysosomal Storage Diseases including antibodies and ELISA kits against NEURONAL CEROID LIPOFUSCINOSIS, GLUCOCEREBROSIDASE, ALPHA-L-IDURONIDASE, AGA, ARSA.

Top Research Reagents

We have 917 products for the study of Lysosomal Storage Diseases 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
Glucosylceramidase/GBA Antibody (2E2)
H00002629-M01
Western Blot: Glucosylceramidase/GBA Antibody (2E2) [H00002629-M01] - Effect of Diltiazem on L444P and N370S/V394L GC Folding and Trafficking.Western blot analysis of untreated and diltiazem-treated L444P GC cells. L444P GC cells were cultured without or with diltiazem at varying concentrations for 7 d before the cells were lysed for SDS-PAGE and Western blot analysis. GC was detected using mouse anti-GC antibody 2E2. Beta-actin served as a loading control. Image collected and cropped by CiteAb from the following publication (//dx.plos.org/10.1371/journal.pbio.0060026) licensed under a CC-BY license.Immunocytochemistry/Immunofluorescence: Glucosylceramidase/GBA Antibody (2E2) [H00002629-M01] - Analysis of monoclonal antibody to GBA on HeLa cell. Antibody concentration 10 ug/ml.

Mouse Monoclonal
Species Human, Plant
Applications WB, ELISA, ICC/IF

     1 Review

10 Publications
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
Sulfamidase/SGSH Antibody
NBP1-83164
Immunohistochemistry-Paraffin: Sulfamidase/SGSH Antibody [NBP1-83164] - Analysis in human adrenal gland and skeletal muscle tissues. Corresponding SGSH RNA-seq data are presented for the same tissues.Immunohistochemistry-Paraffin: Sulfamidase/SGSH Antibody [NBP1-83164] - Staining of human adrenal gland, duodenum, kidney and skeletal muscle using Anti-SGSH antibody (A) NBP1-83164 shows similar protein distribution across tissues to independent antibody NBP1-83165 (B).

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
ASAH1 Antibody
NBP1-89296
Western Blot: ASAH1 Antibody [NBP1-89296] - Analysis in human cell lines SK-MEL-30 and U-251MG using anti-ASAH1 antibody. Corresponding ASAH1 RNA-seq data are presented for the same cell lines. Loading control: anti-HDAC1.Immunohistochemistry-Paraffin: ASAH1 Antibody [NBP1-89296] - Staining of human prostate shows strong granular cytoplasmic positivity in glandular cells.

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

7 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-Galactosidase A/GLA [Unconjugated]
6146-GH
Recombinant Human alpha-Galactosidase A/GLA Protein, CF (Catalog # 6146-GH) hydrolyses the terminal alpha -galactosyl moieties from glycolipids and glycoproteins predominantly ceramide trihexoside.Recombinant Human alpha-Galactosidase A/GLA Protein, CF (Catalog # 6146-GH) is measured by its ability to hydrolyze 4-methylumbelliferyl-alpha -D-galactopyranoside.


Species Human
Applications EnzAct

3 Publications
PSAP Antibody [Unconjugated]
AF8520
Western blot shows lysates of A431 human epithelial carcinoma cell line. PVDF membrane was probed with 1:1000 dilution of Rabbit Anti-Human PSAP Antigen Affinity-purified Polyclonal Antibody (Catalog # AF8520) followed by HRP-conjugated Anti-Rabbit IgG Secondary Antibody (Catalog # <a class=PSAP was detected in immersion fixed A431 human epithelial carcinoma cell line using Rabbit Anti-Human PSAP Antigen Affinity-purified Polyclonal Antibody (Catalog # AF8520) at 1:100 dilution for 3 hours at room temperature. Cells were stained using the NorthernLights™ 557-conjugated Anti-Rabbit IgG Secondary Antibody (red; Catalog # <a class=

Rabbit Polyclonal
Species Human
Applications WB, Simple Western, ICC

     1 Review

2 Publications
PPT1 Antibody (OTI1F10)
NBP2-45388
Western Blot: PPT1 Antibody (1F10) [NBP2-45388] - Analysis of extracts (35ug) from 9 different cell lines by usin g anti-PPT1 monoclonal antibody (HepG2: human; HeLa: human; SVT2: mouse; A549: human; COS7: monkey; Jurkat: human; MDCK: canine; PC12: rat; MCF7: human).Immunohistochemistry-Paraffin: PPT1 Antibody (1F10) [NBP2-45388] - Analysis of Adenocarcinoma of Human breast tissue. (Heat-induced epitope retrieval by 10mM citric buffer, pH6.0, 120C for 3min)

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

2 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
SMPD1 Antibody (OTI3H7)
NBP2-45889
Western Blot: SMPD1 Antibody (3H7) [NBP2-45889] - Analysis of HEK293T cells were transfected with the pCMV6-ENTRY control (Left lane) or pCMV6-ENTRY SMPD1.Immunohistochemistry: SMPD1 Antibody (3H7) [NBP2-45889] - Analysis of Human tonsil tissue. (Heat-induced epitope retrieval by 1 mM EDTA in 10mM Tris, pH8.5, 120C for 3min)

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

4 Publications
Tripeptidyl-Peptidase I/TPP1 Antibody
NBP2-48820
Immunohistochemistry-Paraffin: Tripeptidyl-Peptidase I/TPP1 Antibody [NBP2-48820] - Staining of human kidney.Immunohistochemistry: Tripeptidyl-Peptidase I/TPP1 Antibody [NBP2-48820] - Staining of human heart muscle shows strong granular cytoplasmic positivity in myocytes.

Rabbit Polyclonal
Species Human
Applications IHC, IHC-P

Mucolipin 1 Antibody - BSA Free
NB110-82375
Western Blot: Mucolipin 1 Antibody [NB110-82375] - Total protein from mouse brain was separated on a 7.5% gel by SDS-PAGE, transferred to PVDF membrane and blocked in 5% non-fat milk in TBST. The membrane was probed with 2.0 ug/ml anti-Mucolipin 1 (NB110-82375) in blocking buffer and detected with an anti-rabbit HRP secondary antibody using NovaLume chemiluminescence detection reagent (NPB2-61915).Simple Western: Mucolipin 1 Antibody [NB110-82375] - Lane view shows a specific band for Mucolipin 1 in 0.5 mg/ml of Human Brain lysate. This experiment was performed under reducing conditions using the 12-230 kDa separation system.

Rabbit Polyclonal
Species Human, Mouse, Rat
Applications WB, Simple Western

     1 Review

4 Publications
LYAG/GAA Antibody (2489C) [Unconjugated]
MAB8329
Western blot shows lysates of human placenta tissue, T47D human breast cancer cell line, and MCF-7 human breast cancer cell line. PVDF membrane was probed with 1 µg/mL of Rabbit Anti-Human Lysosomal a-Glucosidase Monoclonal Antibody (Catalog # MAB8329) followed by HRP-conjugated Anti-Rabbit IgG Secondary Antibody (Catalog # <a class= Lysosomal alpha ‑Glucosidase was detected in immersion fixed paraffin-embedded sections of human kidney using Rabbit Anti-Human Lysosomal alpha ‑Glucosidase Monoclonal Antibody (Catalog # MAB8329) at 3 µg/mL for 1 hour at room temperature followed by incubation with the Anti-Rabbit IgG VisUCyte™ HRP Polymer Antibody (Catalog # <a class=

Rabbit Monoclonal
Species Human
Applications WB, IHC


Related Genes

Lysosomal Storage Diseases has been researched against:

Related Pathways

Lysosomal Storage Diseases has been linked to:

Related Diseases

Lysosomal Storage Diseases has been studied in relation to diseases such as:

Related PTMs

Lysosomal Storage Diseases has been studied in relation to posttranslational modifications (PTMs) including:

Alternate Names

Lysosomal Storage Diseases is also known as Disease, Lysosomal Storage, Diseases, Lysosomal Storage, Disorder Of Lysosomal Enzyme, Lysosomal Enzyme Disorders, Lysosomal Storage Disease.