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Zellweger Syndrome: Disease Bioinformatics

Zellweger syndrome is a disease in which one of 13 genes (the PEX genes) has a defect. Zellweger syndrome is one of four diseases in which one of these 13 genes is affected. The group of the four diseases is referred to as PBD (Peroxisome Biogenesis Disorders). Zellweger syndrome is the most severe of the PBD group; meaning that a person suffering from Zellweger syndrome would have the most extreme defect. Defects include wide set eyes, high foreheads, underdeveloped eyebrow ridges, mental retardation, and seizures. There is currently no cure for Zellweger Syndrome and the mortality rate is very high.

Top Research Reagents

We have 1586 products for the study of Zellweger Syndrome that can be applied to Flow Cytometry, Immunocytochemistry/ Immunofluorescence, Immunohistochemistry, Western Blot from our catalog of antibodies and ELISA kits.

PEX10 Antibody - BSA Free
NBP3-18137
Western Blot: PEX10 Antibody - BSA Free [NBP3-18137] - WB Validation in Rat Tissues. Loading: 15 ug of lysates per lane. Antibodies: PEX10 NBP3-18137, 4 ug/mL, 1h incubation at RT in 5% NFDM/TBST. Secondary: Goat anti-rabbit IgG HRP conjugate at 1:10,000 dilution.Immunocytochemistry/Immunofluorescence: PEX10 Antibody - BSA Free [NBP3-18137] - Immunofluorescent analysis of 4% paraformaldehyde-fixed Daudi cells labeling PEX10 with NBP3-18137 at 20 ug/mL, followed by goat anti-rabbit IgG secondary antibody at 1/500 dilution (green) and DAPI staining (blue).

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

PEX7 Antibody
NBP1-57578
Western Blot: PEX7 Antibody [NBP1-57578] - MCF-7 whole cell lysates, concentration 0.2-1 ug/ml.

Rabbit Polyclonal
Species Human
Applications WB

1 Publication
GNPAT Antibody
H00008443-B01P
Western Blot: GNPAT Antibody [H00008443-B01P] - Analysis of GNPAT expression in transfected 293T cell line by GNPAT polyclonal antibody. Lane 1: GNPAT transfected lysate(74.8 KDa). Lane 2: Non-transfected lysate.Immunocytochemistry/Immunofluorescence: GNPAT Antibody [H00008443-B01P] - Analysis of purified antibody to GNPAT on HeLa cell. (antibody concentration 10 ug/ml)

Mouse Polyclonal
Species Human
Applications WB, ICC/IF

PEX1 Antibody
NBP1-80577
Western Blot: PEX1 Antibody [NBP1-80577] - Analysis in human cell line AN3-CA.Immunocytochemistry/Immunofluorescence: PEX1 Antibody [NBP1-80577] - Staining of human cell line A-431 shows positivity in vesicles. Antibody staining is shown in green.

Rabbit Polyclonal
Species Human
Applications WB, ICC/IF

3 Publications
ACOX1 Antibody
NBP1-80950
Immunohistochemistry-Paraffin: ACOX1 Antibody [NBP1-80950] - Staining of human gastrointestinal, kidney, liver and pancreas using Anti-ACOX1 antibody NBP1-80950 (A) shows similar protein distribution across tissues to independent antibody NBP1-80949 (B).Immunocytochemistry/Immunofluorescence: ACOX1 Antibody [NBP1-80950] - Staining of human cell line U-2 OS shows localization to nucleoli & vesicles. Antibody staining is shown in green.

Rabbit Polyclonal
Species Human
Applications ICC/IF, IHC, IHC-P

1 Publication
PEX6 Antibody
NBP1-80955
Immunohistochemistry-Paraffin: PEX6 Antibody [NBP1-80955] - Analysis in human fallopian tube and skeletal muscle tissues. Corresponding PEX6 RNA-seq data are presented for the same tissues.Western Blot: PEX6 Antibody [NBP1-80955] - Lane 1: NIH-3T3 cell lysate (Mouse embryonic fibroblast cells). Lane 2: NBT-II cell lysate (Rat Wistar bladder tumor cells).

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

2 Publications
ZNF260 Antibody
NBP1-80967
Immunocytochemistry/Immunofluorescence: ZNF260 Antibody [NBP1-80967] - Immunofluorescent staining of human cell line A-431 shows localization to nucleoplasm & cytosol.Immunohistochemistry-Paraffin: ZNF260 Antibody [NBP1-80967] - Staining of human stomach shows strong nuclear and additional cytoplasmic positivity in glandular cells.

Rabbit Polyclonal
Species Human
Applications ICC/IF, IHC, IHC-P

PEX13 Antibody
NBP1-86321
Immunohistochemistry-Paraffin: PEX13 Antibody [NBP1-86321] - Staining in human testis and pancreas tissues using anti-PEX13 antibody. Corresponding PEX13 RNA-seq data are presented for the same tissues.Immunohistochemistry-Paraffin: PEX13 Antibody [NBP1-86321] - Staining of human colon, kidney, liver and pancreas using Anti-PEX13 antibody NBP1-86321 (A) shows similar protein distribution across tissues to independent antibody NBP2-38204 (B).

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

PEX5 Antibody
NBP1-87185
Immunohistochemistry-Paraffin: PEX5 Antibody [NBP1-87185] - Staining of human cerebral cortex, colon, lymph node and testis using Anti-PEX5 antibody NBP1-87185 (A) shows similar protein distribution across tissues to independent antibody NBP2-38443 (B).Western Blot: PEX5 Antibody [NBP1-87185] - Analysis using Anti-PEX5 antibody NBP1-87185 (A) shows similar pattern to independent antibody NBP2-38443 (B).

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

4 Publications
PMP70 Antibody
NBP1-87258
Immunohistochemistry-Paraffin: PMP70 Antibody [NBP1-87258] - Staining in human liver and skeletal muscle tissues using NBP1-87258 antibody. Corresponding ABCD3 RNA-seq data are presented for the same tissues.Immunohistochemistry-Paraffin: PMP70 Antibody [NBP1-87258] - Staining of human kidney, liver, skeletal muscle and testis using Anti-ABCD3 antibody NBP1-87258 (A) shows similar protein distribution across tissues to independent antibody NBP2-38199 (B).

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

5 Publications
beta Amyloid Antibody (MOAB-2) - BSA Free
NBP2-13075
IHC analysis of beta Amyloid on normal mouse brain (left) and 5xFAD mouse brain (right) using DAB with hematoxylin counterstain. The MOAB-2 antibody was used at 1:20 on normal mouse brain and at 1:400 on 5xFAD mouse brain.Analysis of beta Amyloid (MOAB-2) antibody in (1) 100 pmole beta Amyloid 42, (2) 5xFAD mouse brain homogenate Repetition 1 and (3) 5xFAD mouse brain homogenate Repetition 2.

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

     2 Reviews

70 Publications
Catalase Antibody [Unconjugated]
AF3398
Western blot shows lysates of Jurkat human acute T cell leukemia cell line, Raji human Burkitt's lymphoma cell line, HeLa human cervical epithelial carcinoma cell line, NIH-3T3 mouse embryonic fibroblast cell line, A20 mouse B cell lymphoma cell line, and Rat-2 rat embryonic fibroblast cell line. PVDF membrane was probed with 0.5 µg/mL of Goat Anti-Human/Mouse/Rat Catalase Antigen Affinity-purified Polyclonal Antibody (Catalog # AF3398) followed by HRP-conjugated Anti-Goat IgG Secondary Antibody (Catalog # <a class=Simple Western lane view shows lysates of Jurkat human acute T cell leukemia cell line and Raji human Burkitt's lymphoma cell line, loaded at 0.2 mg/mL. A specific band was detected for Catalase at approximately 62 kDa (as indicated) using 5 µg/mL of Goat Anti-Human/Mouse/Rat Catalase Antigen Affinity-purified Polyclonal Antibody (Catalog # AF3398) followed by 1:50 dilution of HRP-conjugated Anti-Goat IgG Secondary Antibody (Catalog # <a class=

Goat Polyclonal
Species Human, Mouse, Rat
Applications WB, Simple Western, ICC

     3 Reviews

15 Publications
PHEX
8090-ZN


Species Human
Applications EnzAct

HSD17B4 Antibody (OTI4C4)
NBP2-46005
Western Blot: HSD17B4 Antibody (OTI4C4) [NBP2-46005] - Analysis of HEK293T cells were transfected with the pCMV6-ENTRY control (Left lane) or pCMV6-ENTRY HSD17B4.Immunohistochemistry: HSD17B4 Antibody (OTI4C4) [NBP2-46005] - Analysis of Carcinoma of Human liver tissue.

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

     1 Review

2 Publications
ABCD1 Antibody (OTI2C12)
NBP2-46476
Knockdown Validated: ABCD1 Antibody (OTI2C12) [NBP2-46476] - Western blot shows lysates of TIME human endothelial parental cell line and ABCD1 knockout (KO) TIME cell line. PVDF membrane was probed with 1:2000 of Mouse Anti-Human ABCD1 Monoclonal Antibody (Catalog # NBP2-46476) followed by HRP-conjugated Anti-Mouse IgG Secondary Antibody (Catalog #HAF018). Specific band was detected for ABCD1 at approximately 75 kDa (as indicated) in the parental TIME cell line, but is not detectable in the knockout TIME cell line. This experiment was conducted under reducing conditions.Western Blot: ABCD1 Antibody (OTI2C12) [NBP2-46476] - Analysis of HEK293T cells were transfected with the pCMV6-ENTRY control (Left lane) or pCMV6-ENTRY ABCD1.

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

PEX12 Antibody
NBP2-49599
Immunohistochemistry-Paraffin: PEX12 Antibody [NBP2-49599] - Staining in human epididymis and bone marrow tissues using anti-PEX12 antibody. Corresponding PEX12 RNA-seq data are presented for the same tissues.Western Blot: PEX12 Antibody [NBP2-49599] - Lane 1: Marker [kDa] 250, 130, 95, 72, 55, 36, 28, 17, 10<br/>Lane 2: Human cell line RT-4<br/>Lane 3: Human cell line U-251 MG

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

AAAS Antibody (5A1)
H00008086-M02
Western Blot: AAAS Antibody (5A1) [H00008086-M02] - AAAS monoclonal antibody (M02), clone 5A1. Analysis of AAAS expression in NIH/3T3.Immunohistochemistry-Paraffin: AAAS Antibody (5A1) [H00008086-M02] - Analysis of monoclonal antibody to AAAS on formalin-fixed paraffin-embedded human prostate. Antibody concentration 3 ug/ml.

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

DAO Antibody - Azide and BSA Free
NBP2-92892
Western Blot: DAO Antibody [NBP2-92892] - Analysis of extracts of Rat kidney, using DAO antibody at 1:3000 dilution.Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) at 1:10000 dilution.Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST.Detection: ECL Basic Kit. Exposure time: 1s.Immunocytochemistry/Immunofluorescence: DAO Antibody [NBP2-92892] - Analysis of L929 cells using DAO at dilution of 1:100. Blue: DAPI for nuclear staining.

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

     1 Review

1 Publication
PXMP3 Antibody - Azide and BSA Free
NBP2-94643
Western Blot: PXMP3 Antibody [NBP2-94643] - Analysis of extracts of various cell lines, using PXMP3 at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit . Exposure time: 20s.Immunohistochemistry-Paraffin: PXMP3 Antibody [NBP2-94643] - Immunohistochemistry of paraffin-embedded human placenta using PXMP3 Rabbit pAb (NBP2-94643) at dilution of 1:100 (40x lens). Perform high pressure antigen retrieval with 10 mM citrate buffer pH 6.0 before commencing with IHC staining protocol.

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


Related Genes

Zellweger Syndrome has been researched against:

Related Pathways

Zellweger Syndrome has been linked to:

Related Diseases

Zellweger Syndrome has been studied in relation to diseases such as:

Related PTMs

Zellweger Syndrome has been studied in relation to posttranslational modifications (PTMs) including:

Alternate Names

Zellweger Syndrome is also known as zellweger syndrome, zellweger syndrome (disorder), cerebrohepatorenal syndrome, zellweger leukodystrophy, congenital iron overload, zws, chr, zes, zs.