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Congenital Structural Myopathy: Disease Bioinformatics

Research of Congenital Structural Myopathy has been linked to Myopathy, Weakness, Congenital Myopathy (disorder), Muscle Hypotonia, Muscle Weakness. The study of Congenital Structural Myopathy has been mentioned in research publications which can be found using our bioinformatics tool below. Researched pathways related to Congenital Structural Myopathy include Pathogenesis, Localization, Endocytosis, Innervation, Muscle Atrophy. These pathways complement our catalog of research reagents for the study of Congenital Structural Myopathy including antibodies and ELISA kits against INTERMEDIATE FILAMENT, ACTA1, BIN1, DES, DMD.

Top Research Reagents

We have 1728 products for the study of Congenital Structural Myopathy that can be applied to Flow Cytometry, Immunocytochemistry/ Immunofluorescence, Immunohistochemistry, Western Blot from our catalog of antibodies and ELISA kits.

Actin Antibody (mAbGEa) - BSA Free
NB100-74340
Western Blot: Actin Antibody (mAbGEa) [NB100-74340] - Total protein from HeLa, 3T3, PC12 and Bovine normal tissue was separated on a 12% gel by SDS-PAGE, transferred to PVDF membrane and blocked in 5% non-fat milk in TBST. The membrane was probed with 1.0 ug/mL anti-Actin in 5% blocking buffer and detected with an anti-mouse IgM secondary antibody using chemiluminescence.Immunocytochemistry/Immunofluorescence: Actin Antibody (mAbGEa) [NB100-74340] - NIH-3T3 cells were fixed for 10 minutes using 10% formalin and then permeabilized for 5 minutes using 1X PBS + 0.05% Triton X-100. The cells were incubated with anti-Actin (mAbGEa) at 5 ug/mL overnight at 4C and detected with an anti-Mouse IgM DyLight 488 (Green) at a 1:500 dilution. Nuclei were counterstained with DAPI (Blue). Cells were imaged using a 40X objective.

Mouse Monoclonal
Species Human, Mouse, Rat
Applications WB, Simple Western, ELISA

     8 Reviews

34 Publications
Vimentin Antibody
NB300-223
Western Blot: Vimentin Antibody [NB300-223] - Analysis of tissue and cell lysates. Antibody at 1:5000 in red. [1] protein standard (red), [2] rat whole brain lysate, [3] HeLa, [4] SH-SY5Y, [5] HEK293, and [6] NIH-3T3 cell lysates. NB300-223 binds to the vimentin protein showing a single band at ~50 kDa. The blot was simultaneously probed with mouse mAb to MAP2C/D, dilution 1:5000 in green, revealing multiple bands around 280 kDa that correspond to full length MAP2A/2B isotypes, and ~70 kDa bands which are MAP2C/D isotypes. MAP2 isotypes are seen only in extracts containing neuronal lineage cells.Immunohistochemistry: Vimentin Antibody [NB300-223] - Attenuated reactive astrocytosis after stroke in Smad1 cKO mice. Enlarged IHC images of boxed areas at the cortical peri-infarct area with the indicated reactive astrocyte markers GFAP, Nestin, and Vimentin. Enlarged images of GFAP IHC highlight the hypertrophic morphology of GFAP+ astrocytes in mutants. Image collected and cropped by CiteAb from the following publication (https://dx.plos.org/10.1371/journal.pone.0136967), licensed under a CC-BY license.

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

     6 Reviews

80 Publications
Ryanodine Receptor Antibody (34C) - BSA Free
NB300-543
Immunohistochemistry-Paraffin: Ryanodine Receptor Antibody (34C) [NB300-543] - Biopsies of deparaffinized Human skeletal muscle tissue.

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

12 Publications
MTMR1 Antibody - BSA Free
NBP1-86042
Western Blot: MTMR1 Antibody [NBP1-86042] - Lane 1: Marker [kDa] 230, 130, 95, 72, 56, 36, 28, 17, 11. Lane 2: Human cell line RT-4. Lane 3: Human cell line U-251MG spImmunohistochemistry-Paraffin: MTMR1 Antibody [NBP1-86042] - Staining of human smooth muscle shows cytoplasmic positivity in smooth muscle cells.

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

Secretin R Antibody - BSA Free
NBP1-86125
Immunohistochemistry-Paraffin: Secretin R Antibody [NBP1-86125] - Staining of human stomach shows strong membranous positivity in glandular cells.Immunohistochemistry-Paraffin: Secretin R Antibody [NBP1-86125] - Staining of human pancreas shows strong membranous positivity in interlobular ducts.

Rabbit Polyclonal
Species Human
Applications IHC, IHC-P

1 Publication
BIN1 Antibody - BSA Free
NBP1-89102
Western Blot: BIN1 Antibody [NBP1-89102] - Analysis in human cell lines Caco-2 and MCF-7 using anti-BIN1 antibody. Corresponding BIN1 RNA-seq data are presented for the same cell lines. Loading control: anti-PPIB.Immunocytochemistry/Immunofluorescence: BIN1 Antibody [NBP1-89102] - Staining of human cell line U-2 OS shows localization to cytosol. Antibody staining is shown in green.

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

2 Publications
Dystrophin Antibody - BSA Free
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
gamma-Synuclein Antibody - BSA Free
NBP1-89996
Western Blot: gamma-Synuclein Antibody [NBP1-89996] - Lane 1: Marker [kDa] 230, 130, 95, 72, 56, 36, 28, 17, 11. Lane 2: Human cell line RT-4Immunohistochemistry-Paraffin: gamma-Synuclein Antibody [NBP1-89996] - Staining of human urinary bladder shows strong cytoplasmic positivity in urothelial cells.

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

SBF2 Antibody - BSA Free
NBP2-13283
Western Blot: SBF2 Antibody [NBP2-13283] - Analysis in human cell line BEWO.Immunohistochemistry-Paraffin: SBF2 Antibody [NBP2-13283] - Staining of human prostate shows weak cytoplasmic positivity in smooth muscle cells.

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

SEPN1 Antibody - BSA Free
NBP2-34098
Immunocytochemistry/Immunofluorescence: SEPN1 Antibody [NBP2-34098] - Immunofluorescent staining of human cell line A-431 shows localization to cytosol.Immunohistochemistry-Paraffin: SEPN1 Antibody [NBP2-34098] - Staining of human lung shows strong cytoplasmic positivity in pneumocytes and macrophages.

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

Desmin Antibody [Unconjugated]
AF3844
Desmin was detected in immersion fixed paraffin-embedded sections of human heart using 15 µg/mL Human Desmin Antigen Affinity-purified Polyclonal Antibody (Catalog # AF3844) overnight at 4 °C. Tissue was stained with the Anti-Goat HRP-DAB Cell & Tissue Staining Kit (brown; Catalog # <a class=Desmin was detected in immersion fixed frozen sections of mouse skeletal muscle using Human Desmin Antigen Affinity-purified Polyclonal Antibody (Catalog # AF3844) at 10 µg/mL overnight at 4 °C. Tissue was stained using the NorthernLights™ 557-conjugated Anti-Goat IgG Secondary Antibody (red; Catalog # <A class=NoLineLink href=

Goat Polyclonal
Species Human, Mouse
Applications IHC

     2 Reviews

34 Publications
Myosin Heavy Chain Antibody (MF20) [Unconjugated]
MAB4470
Myosin Heavy Chain was detected in immersion fixed C2C12 mouse myoblast cell line using Mouse Anti-Human Myosin Heavy Chain Monoclonal Antibody (Catalog # MAB4470) 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= Myosin Heavy Chain was detected in immersion fixed paraffin-embedded sections of human skeletal muscle using Mouse Anti-Myosin Heavy Chain Monoclonal Antibody (Catalog # MAB4470) at 5 µg/mL for 1 hour at room temperature followed by incubation with the Anti-Mouse IgG VisUCyte™ HRP Polymer Antibody (Catalog # <a class=

Mouse Monoclonal
Species Multi-Species
Applications WB, Flow, IHC

     1 Review

166 Publications
Dynamin 2 Antibody - BSA Free
NBP2-47477
Immunohistochemistry-Paraffin: Dynamin 2 Antibody [NBP2-47477] - Analysis in human colon and liver tissues. Corresponding Dynamin 2 RNA-seq data are presented for the same tissues.Western Blot: Dynamin 2 Antibody [NBP2-47477] - Lane 1: Marker [kDa] 250, 130, 95, 72, 55, 36, 28, 17, 10. Lane 2: Human cell line RT-4. Lane 3: Human cell line U-251MG.

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

3 Publications
Dysferlin Antibody (JF05-54)
NBP2-66814
Western Blot: Dysferlin Antibody (JF05-54) [NBP2-66814] - Analysis of Dysferlin on human skeletal muscle lysates using anti-Dysferlin antibody at 1/1,000 dilution.Immunocytochemistry/Immunofluorescence: Dysferlin Antibody (JF05-54) [NBP2-66814] - Staining Dysferlin in SW480 cells (red). The nuclear counter stain is DAPI (blue). Cells were fixed in paraformaldehyde, permeabilised with 0.25% Triton X100/PBS.

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

MTMR14 Antibody (OTI6B6) - Azide and BSA Free
NBP2-72808
Western Blot: MTMR14 Antibody (OTI6B6) - Azide and BSA Free [NBP2-72808] - HEK293T cells were transfected with the pCMV6-ENTRY control (Left lane) or pCMV6-ENTRY MTMR14 (Right lane) cDNA for 48 hrs and lysed. Equivalent amounts of cell lysates (5 ug per lane) were separated by SDS-PAGE and immunoblotted with anti-MTMR14.Flow Cytometry: MTMR14 Antibody (OTI6B6) - Azide and BSA Free [NBP2-72808] - Staining of COS7 cells transiently transfected by pCMV6-ENTRY MTMR14.

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

Myotubularin Antibody
NB300-949
Western Blot: Myotubularin Antibody [NB300-949] - Staining of Mouse Skeletal Muscle lysate (35 ug protein in RIPA buffer). Antibody at 1 ug/mL. Detected by chemiluminescence.Immunohistochemistry-Paraffin: Myotubularin Antibody [NB300-949] - Staining of paraffin embedded Human Uterus. Antibody at 10 ug/mL. Steamed antigen retrieval with citrate buffer pH 6, AP-staining.

Goat Polyclonal
Species Human, Mouse
Applications WB, IHC, IHC-P

1 Publication
MTMR2 Antibody (4G6) - Azide and BSA Free
H00008898-M03
Western Blot: MTMR2 Antibody (4G6) [H00008898-M03] - Analysis of MTMR2 expression in PC-12 (Cat # L012V1).Western Blot: MTMR2 Antibody (4G6) [H00008898-M03] - Analysis of MTMR2 expression in HeLa (Cat # L013V1).

Mouse Monoclonal
Species Human, Rat
Applications WB, ELISA

1 Publication
SBF1 Antibody - Azide and BSA Free
NBP2-93980
Western Blot: SBF1 Antibody [NBP2-93980] - Analysis of extracts of various cell lines, using SBF1 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: 90s.

Rabbit Polyclonal
Species Human, Rat
Applications WB, ELISA

tropomyosin-3 Antibody (2E4) - Azide and BSA Free
H00007170-M02
Western Blot: tropomyosin-3 Antibody (2E4) [H00007170-M02] - Enrichment of tumor proteins in plasma-derived EVs and classifier performance. Immunoblot analysis of plasma-derived extracellular vesicles from adenocarcinoma cases and matched controls to demonstrate marker performance in immunoassay. Marker abundance as quantified by densitometry of western blot, lower left; ROC curve analysis of TPM3 performance based on western blot data, lower right. Image collected and cropped by CiteAb from the following publication (//www.oncotarget.com/fulltext/20748) licensed under a CC-BY license.Immunohistochemistry-Paraffin: tropomyosin-3 Antibody (2E4) [H00007170-M02] - Analysis of monoclonal antibody to TPM3 on formalin-fixed paraffin-embedded human smooth muscle. Antibody concentration 3 ug/ml.

Mouse Monoclonal
Species Human
Applications WB, ELISA, IHC

2 Publications

Related Genes

Congenital Structural Myopathy has been researched against:

Related Pathways

Congenital Structural Myopathy has been linked to:

Related Diseases

Congenital Structural Myopathy has been studied in relation to diseases such as:

Related PTMs

Congenital Structural Myopathy has been studied in relation to posttranslational modifications (PTMs) including:

Alternate Names

Congenital Structural Myopathy is also known as congenital structural myopathy, myopathies, structural, congenital, myopathy.