catalog number :
MBS248269
products full name :
Rabbit Polyclonal to Human DES / Desmin
products short name :
[DES / Desmin]
products name syn :
[DES; ARVC7; ARVD7; CMD1I; CSM1; CSM2; MFM1; SCPNK; Desmin; Intermediate filament protein]
other names :
[desmin; Desmin; desmin; mutant desmin p.K241E; intermediate filament protein; desmin]
products gene name :
[DES]
other gene names :
[DES; DES; CSM1; CSM2; LGMD2R]
uniprot entry name :
DESM_HUMAN
reactivity :
Human, Mouse, Rat
specificity :
Human Desmin
purity :
Immunoaffinity Purified
form :
PBS, pH 7.2, 15 mM Sodium Azide
storage stability :
Short term: store at 4°C. Long term: aliquot and store at -20°C. Avoid freeze-thaw cycles.
tested application :
Immunohistochemistry (IHC), Immunohistochemistry (IHC - Paraffin), Immunofluorescence (IF), Western Blot (WB)
app notes :
IHC-Paraffin (1:200). Immunofluorescence (1:50 - 1:200). Western blot (1:500 - 1:1000). Optimal dilution to be determined by the researcher.
image1 heading :
Immunohistochemistry (IHC)
image2 heading :
Western Blot (WB)
other info1 :
Host Note: DES / Desmin antibody was produced in Rabbit. Immunogen Species: DES / Desmin antibody was raised against Human. Antigen Type: Synthetic peptide. Immunogen: DES / Desmin antibody was raised against synthetic peptide around Ile448 of human Desmin. Epitope: Ile448
other info2 :
. Disclaimer: Due to the highly specific nature of antibodies and antigens, we cannot predict or be held responsible with respect to how this antibody will behave in your systems. Researchers using this antibody should conduct optimization studies to achieve the most optimal result possible for their intended application. Recommended Immunohistochemistry Protocol: The following protocol is a recommendation only, and MyBioSource, Inc. makes no guarantee of the results:. Tissue Preparation: . Formalin fixation and embedding in paraffin wax. Tissue Sectioning: . Make 4-um sections and place on pre-cleaned and charged microscope slides. Heat in a tissue-dryingoven for 45 minutes at 60°C. Deparaffinization: . Wash dry slides in 3 changes of xylene - 5 minutes each @ RT. Rehydration: . Wash slides in 3 changes of 100% alcohol - 3 minutes each @ RT. Wash slides in 2 changes of 95% alcohol - 3 minutes each @ RT. Wash slides in 1 change of 80% alcohol - 3 minutes @ RT. Rinse slides in gentle running distilled water - 5 minutes @ RT. Antigen retrieval: . Steam slides in 0.01 M sodium citrate buffer, pH 6.0 at 99-100°C - 20 minutes. Remove from heat and let stand at room temperature in buffer - 20 minutes. Rinse in 1X TBS with Tween (TBST) -1 minute @ RT. Immunostaining: . (Do not allow tissues to dry at any time during the staining procedure) . Apply a universal protein block - 20 minutes @ RT. Drain protein block from slides, apply diluted primary antibody - 45 minutes @ RT. Rinse slides in 1 X TBST - 1 minute @ RT. Apply a biotinylated secondary antibody appropriate for the primary antibody - 30 minutes @ RT. Rinse slides in 1X TBST -1 minute @ RT. Apply alkaline phosphatase streptavidin - 30 minutes @ RT. Rinse slides in 1X TBST -1 minute @ RT. Apply alkaline phosphatase chromogen substrate - 30 minutes @ RT. Wash slides in distilled water - 1 minute @ RT. Dehydrate: . (This method should only be used if the chromogen substrate is alcohol insoluble (e.g. Vector Red, DAB) . Wash slides in 2 changes of 80% alcohol - 1 minute each @ RT. Wash slides in 2 changes of 95% alcohol - 1 minute each @ RT. Wash slides in 3 changes of 100% alcohol - 1 minute each @ RT. Wash slides in 3 changes of xylene - 1 minute each @ RT. Apply coverslip
ncbi acc num :
NP_001918.3
ncbi gb acc num :
NM_001927.3
ncbi pathways :
Arrhythmogenic Right Ventricular Cardiomyopathy Pathway (672454); Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Pathway (117293); Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Pathway (116129); Aurora B Signaling Pathway (138080); Dilated Cardiomyopathy Pathway (121494); Dilated Cardiomyopathy Pathway (121285); Hypertrophic Cardiomyopathy (HCM) Pathway (114229); Hypertrophic Cardiomyopathy (HCM) Pathway (106591); Muscle Contraction Pathway (106261); Striated Muscle Contraction Pathway (198903)
ncbi summary :
This gene encodes a muscle-specific class III intermediate filament. Homopolymers of this protein form a stable intracytoplasmic filamentous network connecting myofibrils to each other and to the plasma membrane. Mutations in this gene are associated with desmin-related myopathy, a familial cardiac and skeletal myopathy (CSM), and with distal myopathies. [provided by RefSeq, Jul 2008]
uniprot summary :
desmin: a class-III intermediate filaments found in muscle cells. In adult striated muscle they form a fibrous network connecting myofibrils to each other and to the plasma membrane from the periphery of the Z-line structures. Mutations in this gene are associated with desmin-related myopathy, a familial cardiac and skeletal myopathy (CSM), and with distal myopathies. Protein type: Motility/polarity/chemotaxis; Cytoskeletal. Chromosomal Location of Human Ortholog: 2q35. Cellular Component: intermediate filament; fascia adherens; Z disc; cytosol; neuromuscular junction; sarcolemma. Molecular Function: identical protein binding; protein binding; structural constituent of cytoskeleton; cytoskeletal protein binding. Biological Process: muscle contraction; cytoskeleton organization and biogenesis; regulation of heart contraction; muscle filament sliding. Disease: Cardiomyopathy, Dilated, 1i; Scapuloperoneal Syndrome, Neurogenic, Kaeser Type; Myopathy, Myofibrillar, 1; Muscular Dystrophy, Limb-girdle, Type 2r