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Muscular Dystrophy, Emery-dreifuss: Disease Bioinformatics

Emery-Dreifuss muscular dystrophy is a disorder that causes muscles weakness primarily in skeletal and cardiac muscles. In most cases, Emery-Dreifuss muscular dystrophy is caused by mutations in the X-linked EMD gene, so this condition affects more males than females. Early symptoms of Emery-Dreifuss muscular dystrophy include joint deformities involving the elbows, ankles, and neck, and progressive muscles weakening. Most individuals with the disorder have heart complications by the time they reach adulthood. There is no cure or prevention technique for Emery-Dreifuss muscular dystrophy, but the disorder can be managed with physical therapy and can be improved with gene therapy.

Top Research Reagents

We have 2010 products for the study of Muscular Dystrophy, Emery-dreifuss that can be applied to Chromatin Immunoprecipitation (ChIP), Flow Cytometry, Immunocytochemistry/ Immunofluorescence, Immunohistochemistry, Western Blot from our catalog of antibodies and ELISA kits.

Emerin Antibody
NBP1-87692
Western Blot: Emerin Antibody [NBP1-87692] - Analysis in RT-4 cells transfected with control siRNA, target specific siRNA probe #1 and #2. Remaining relative intensity is presented. Loading control: Anti-GAPDH.Immunocytochemistry/Immunofluorescence: Emerin Antibody [NBP1-87692] - Staining of human cell line U-251 MG shows localization to nuclear membrane & endoplasmic reticulum. Antibody staining is shown in green.

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

5 Publications
Thymopoietin/LAP2 Antibody
NBP1-87822
Western Blot: Thymopoietin/LAP2 Antibody [NBP1-87822] - Analysis in mouse cell line NIH-3T3 and rat cell line NBT-II.Immunocytochemistry/Immunofluorescence: Thymopoietin/LAP2 Antibody [NBP1-87822] - Staining of human cell line A-431 shows localization to nuclear membrane. Antibody staining is shown in green.

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

2 Publications
SYNE1 Antibody
NBP1-89349
Immunocytochemistry/Immunofluorescence: SYNE1 Antibody [NBP1-89349] - Staining of human cell line U-251 MG shows localization to nucleoplasm & nuclear membrane. Antibody staining is shown in green.Immunohistochemistry-Paraffin: SYNE1 Antibody [NBP1-89349] - Staining of human skeletal muscle shows strong cytoplasmic and nuclear membranous positivity in myocytes.

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

1 Publication
Dystrophin Antibody
NBP1-89953
Immunohistochemistry-Paraffin: Dystrophin Antibody [NBP1-89953] - Staining in human skeletal muscle and tonsil tissues . Corresponding DMD RNA-seq data are presented for the same tissues.Immunohistochemistry-Paraffin: Dystrophin Antibody [NBP1-89953] - Staining of human heart muscle shows strong membranous positivity in myocytes.

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

4 Publications
LEMD3 Antibody (OTI6C10)
NBP2-02211
Western Blot: LEMD3 Antibody (OTI6C10) [NBP2-02211] - Analysis of extracts (35ug) from 9 different cell lines by using anti-LEMD3 monoclonal antibody.Immunocytochemistry/Immunofluorescence: LEMD3 Antibody (OTI6C10) [NBP2-02211] - Staining of COS7 cells transiently transfected by pCMV6-ENTRY LEMD3.

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

Lamin A + C Antibody (4C4)
NBP2-25151
Western Blot: Lamin A + C Antibody (4C4) [NBP2-25151] - Analysis of different cell lysates using mouse mAb to lamin A/C, NBP2-25151, dilution 1:1,000 in green: [1] protein standard (red), [2] HeLa, [3] HEK293 [4] C6, and [5] NIH-3T3 cell lysates. Two strong bands at 74 and 65kDa correspond to the lamin A and lamin C proteins respectively, detected only in the cells of human origin. NBP2-25151 antibody failed to recognize rat or mouse proteins.Immunocytochemistry/Immunofluorescence: Lamin A + C Antibody (4C4) [NBP2-25151] - Analysis of HeLa cells stained with mouse mAb to lamin A/C, NBP2-25151, dilution 1:2,000 in red, and costained with rabbit pAb to HSP60, dilution 1:5,000, in green. The blue is Hoechst staining of nuclear DNA. NBP2-25151 antibody specifically labels the nuclear lamina, while the HSP60 antibody reveals protein expressed in mitochondria.

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

2 Publications
Erbin Antibody [Unconjugated]
AF7866
Western blot shows lysates of A172 human glioblastoma cell line and HeLa human cervical epithelial carcinoma cell line. PVDF membrane was probed with 0.2 µg/mL of Sheep Anti-Human Erbin Antigen Affinity-purified Polyclonal Antibody (Catalog # AF7866) followed by HRP-conjugated Anti-Sheep IgG Secondary Antibody (Catalog # <a class=Erbin was detected in immersion fixed DU145 human prostate carcinoma cell line using Sheep Anti-Human Erbin Antigen Affinity-purified Polyclonal Antibody (Catalog # AF7866) at 10 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, ICC

     2 Reviews

1 Publication
Insulin Antibody (182410) [Unconjugated]
MAB1417
Insulin was detected in immersion fixed beta TC-6 mouse beta cell insulinoma cell line using Human/Mouse/Bovine Insulin Monoclonal Antibody (Catalog # MAB1417) at 10 µg/mL for 3 hours at room temperature. Cells were stained using the NorthernLights™ 557-conjugated Anti-Rat IgG Secondary Antibody (red; Catalog # <a class=Insulin was detected in immersion fixed paraffin-embedded sections of human pancreas using Rat Anti-Human/Mouse/Bovine Insulin Monoclonal Antibody (Catalog # MAB1417) at 0.5 µg/mL for 1 hour at room temperature followed by incubation with the Anti-Rat IgG VisUCyte™ HRP Polymer Antibody (<a class=

Rat Monoclonal
Species Human, Mouse, Bovine
Applications IHC, CyTOF-ready, ICC

25 Publications
Nesprin 2 Antibody
NBP2-42886
Western Blot: Nesprin 2 Antibody [NBP2-42886] - Various tissue extracts (50 ug) were separated by 5% SDS-PAGE, and the membrane was blotted with Nesprin 2 antibody [C3], C-term diluted at 1:10000. The HRP-conjugated anti-rabbit IgG antibody (NBP2-19301) was used to detect the primary antibody, and the signal was developed with Trident ECL plus-Enhanced.Immunohistochemistry-Paraffin: Nesprin 2 Antibody [NBP2-42886] - Rat brain. Nesprin 2 stained by Nesprin 2 antibody [C3], C-term diluted at 1:500.Antigen Retrieval: Citrate buffer, pH 6.0, 15 min.

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

Lamin B1 Antibody (CL3929)
NBP2-59783
Western Blot: Lamin B1 Antibody (CL3929) [NBP2-59783] - Analysis in human cell line MCF-7.Immunocytochemistry/Immunofluorescence: Lamin B1 Antibody (CL3929) [NBP2-59783] - Staining of A-431 cells showing specific staining of nuclear membrane in green. Microtubule and nuclear probes are visualized in red and blue, respectively (where available). Antibody staining is shown in green.

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

     1 Review

7 Publications
Neutrophil Elastase/ELA2 [Biotin]
DY9167-05
N/A Neutrophil Elastase/ELA2 [Biotin]


Species Human
Applications ELISA

23 Publications
Lamin B2 Antibody (2E2F4) - BSA Free
NBP2-61818
Western Blot: Lamin B2 Antibody (2E2F4) [NBP2-61818] - Analysis using LMNB2 mAb against HEK293 (1) and LMNB2 (AA: 401-600)-hIgGFc transfected HEK293 (2) cell lysate.Immunocytochemistry/Immunofluorescence: Lamin B2 Antibody (2E2F4) [NBP2-61818] - Analysis of HepG2 cells using LMNB2 mouse mAb (green). Blue: DRAQ5 fluorescent DNA dye. Red: Actin filaments have been labeled with Alexa Fluor- 555 phalloidin. Goat anti-Mouse IgG (H+L) DyLight 488 secondary antibody was used.

Mouse Monoclonal
Species Human
Applications WB, ELISA, Flow

UNC84B Antibody
NBP2-62573
Western Blot: UNC84B Antibody [NBP2-62573] - Analysis in A-431 cells transfected with control siRNA, target specific siRNA probe #1, using Anti-UNC84B antibody. Remaining relative intensity is presented. Loading control: Anti-GAPDH.Immunohistochemistry-Paraffin: UNC84B Antibody [NBP2-62573] - Staining of human kidney shows moderate to strong positivity in nuclear membrane cells in tubules and cells in glomeruli.

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

HSP90 alpha Antibody
NB120-2928
Western Blot: HSP90 alpha Antibody [NB120-2928] - Analysis of 2-fold serial dilutions of HeLa cell lysate, starting at 10 ug, per well.Immunocytochemistry/Immunofluorescence: HSP90 alpha Antibody [NB120-2928] - Analysis of Heat Shock Protein 86 (HSP86, green) in HeLa cells and NIH3T3 cells. Formalin fixed cells were permeabilized with 0.1% Triton X-100 in TBS for 10 minutes at room temperature and blocked with 1% Blocker BSA for 15 minutes at room temperature. Cells were probed with a HSP86 polyclonal antibody, at a dilution of 1:100 for at least 1 hour at room temperature, washed with PBS, and incubated with DyLight 488 goat-anti-rabbit IgG secondary antibody at a dilution of 1:400 for 30 minutes at room temperature. Nuclei (blue) were stained with Hoechst 33342 dye.

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

13 Publications
BANF1 Antibody (3F10-4G12)
H00008815-M01
Western Blot: BANF1 Antibody (3F10-4G12) [H00008815-M01] - BANF1 monoclonal antibody (M01), clone 3F10-4G12 Analysis of BANF1 expression in Jurkat.Immunohistochemistry-Paraffin: BANF1 Antibody (3F10-4G12) [H00008815-M01] - Analysis of monoclonal antibody to BANF1 on formalin-fixed paraffin-embedded human esophagus. Antibody concentration 3 ug/ml.

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

6 Publications
Lamin B Receptor Antibody
H00003930-B01P
Western Blot: Lamin B Receptor Antibody [H00003930-B01P] - Analysis of LBR expression in transfected 293T cell line by LBR polyclonal antibody. Lane 1: LBR transfected lysate(67.65 KDa). Lane 2: Non-transfected lysate.Immunocytochemistry/Immunofluorescence: Lamin B Receptor Antibody [H00003930-B01P] - Analysis of purified antibody to LBR on HeLa cell. (antibody concentration 10 ug/ml)

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

     1 Review