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Rabbit Anti-Desmin antibody (bsm-60266R)
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说明书: 50ul  100ul  
50ul/1580.00元
100ul/2680.00元
大包装/询价

产品编号 bsm-60266R
英文名称 Desmin
中文名称 结蛋白重组兔单克隆抗体
别    名 CMD1I; CSM1; CSM2; DES; FLJ12025; FLJ39719; FLJ41013; FLJ41793; Intermediate filament protein; OTTHUMP00000064865; DESM_HUMAN; FLJ12025; FLJ39719; FLJ41013; FLJ41793.   
研究领域 肿瘤  心血管  免疫学  信号转导  
抗体来源 Rabbit
克隆类型 Monoclonal
交叉反应 Human, Mouse, Rat, 
产品应用 WB=1:500-2000 IHC-P=1:200-1:1000 ICC=1:50 IF=1:100-500 (石蜡切片需做抗原修复)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 52kDa
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Desmin 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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]

Function:
Desmin are 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.

Subunit:
Homopolymer. Interacts with DST. Interacts with MTM1.

Subcellular Location:
Cytoplasm.

Post-translational modifications:
ADP-ribosylation prevents ability to form intermediate filaments.

DISEASE:
Defects in DES are the cause of myopathy myofibrillar type 1 (MFM1) [MIM:601419]. A neuromuscular disorder characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and by myofibrillar destruction with intracytoplasmic accumulation of desmin-reactive deposits in cardiac and skeletal muscle cells. Note=Mutations in the DES gene are associated with a variable clinical phenotype which encompasses isolated myopathies, pure cardiac phenotypes (including dilated cardiomyopathy, restrictive cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy), cardiac conduction disease, and combinations of these disorders. If both cardiologic and neurologic features occur, they can manifest in any order, as cardiologic features can precede, occur simultaneously with, or follow manifestation of generalized neuromuscular disease (PubMed:19879535).
Defects in DES are the cause of cardiomyopathy dilated type 1I (CMD1I) [MIM:604765]. Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.
Defects in DES are the cause of neurogenic scapuloperoneal syndrome Kaeser type (Kaeser syndrome) [MIM:181400]. Kaeser syndrome is an autosomal dominant disorder with a peculiar scapuloperoneal distribution of weakness and atrophy. A large clinical variability is observed ranging from scapuloperoneal, limb grindle and distal phenotypes with variable cardiac or respiratory involvement. Facial weakness, dysphagia and gynaecomastia are frequent additional symptoms. Affected men seemingly bear a higher risk of sudden, cardiac death as compared to affected women. Histological and immunohistochemical examination of muscle biopsy specimens reveal a wide spectrum of findings ranging from near normal or unspecific pathology to typical, myofibrillar changes with accumulation of desmin.

Similarity:
Belongs to the intermediate filament family.

SWISS:
P17661

Gene ID:
1674

Database links:

Entrez Gene: 1674 Human

Entrez Gene: 13346 Mouse

Entrez Gene: 64362 Rat

SwissProt: P17661 Human

SwissProt: P31001 Mouse

SwissProt: P48675 Rat



产品图片
Blocking buffer: 5% NFDM/TBST
Primary ab dilution: 1:2000
Primary ab incubation condition: 2 hours at room temperature
Lysate: C2C12, Rat heart
Protein loading quantity: 20 μg
Exposure time: 3 s
Predicted MW: 53 kDa
Observed MW: 53 kDa
Tissue: mouse stomach
Section type: Formalin fixed & Paraffin -embedded section
Retrieval method: High temperature and high pressure
Retrieval buffer: Tris/EDTA buffer, pH 9.0 Primary ab dilution: 1:1000
Primary ab incubation condition: 1 hour at room temperature
Counter stain: Hematoxylin
Comment: Color brown is the positive signal for bsm-60266R
Tissue: mouse colon
Section type: Formalin fixed & Paraffin -embedded section
Retrieval method: High temperature and high pressure
Retrieval buffer: Tris/EDTA buffer, pH 9.0 Primary ab dilution: 1:1000
Primary ab incubation condition: 1 hour at room temperature
Counter stain: Hematoxylin
Comment: Color brown is the positive signal for bsm-60266R
Tissue: rat stomach
Section type: Formalin fixed & Paraffin -embedded section
Retrieval method: High temperature and high pressure
Retrieval buffer: Tris/EDTA buffer, pH 9.0 Primary ab dilution: 1:1000
Primary ab incubation condition: 1 hour at room temperature
Counter stain: Hematoxylin
Comment: Color brown is the positive signal for bsm-60266R
Tissue: human endometrial
Section type: Formalin fixed & Paraffin -embedded section
Retrieval method: High temperature and high pressure
Retrieval buffer: Tris/EDTA buffer, pH 9.0 Primary ab dilution: 1:1000
Primary ab incubation condition: 1 hour at room temperature
Counter stain: Hematoxylin
Comment: Color brown is the positive signal for bsm-60266R
Tissue: mouse skeletal muscle
Section type: Formalin fixed & Paraffin -embedded section
Retrieval method: High temperature and high pressure
Retrieval buffer: Tris/EDTA buffer, pH 9.0 Primary ab dilution: 1:1000
Primary ab incubation condition: 1 hour at room temperature
Counter stain: Hematoxylin
Comment: Color brown is the positive signal for bsm-60266R
Tissue: mouse myocardium
Section type: Formalin fixed & Paraffin -embedded section
Retrieval method: High temperature and high pressure
Retrieval buffer: Tris/EDTA buffer, pH 9.0 Primary ab dilution: 1:1000
Primary ab incubation condition: 1 hour at room temperature
Counter stain: Hematoxylin
Comment: Color brown is the positive signal for bsm-60266R
Tissue: Human esophageal
Section type: Formalin fixed & Paraffin -embedded section
Retrieval method: High temperature and high pressure
Retrieval buffer: Tris/EDTA buffer, pH 9.0 Primary ab dilution: 1:1000
Primary ab incubation condition: 1 hour at room temperature
Counter stain: Hematoxylin
Comment: Color brown is the positive signal for bsm-60266R
Tissue: rat skeletal muscle
Section type: Formalin fixed & Paraffin -embedded section
Retrieval method: High temperature and high pressure
Retrieval buffer: Tris/EDTA buffer, pH 9.0 Primary ab dilution: 1:1000
Primary ab incubation condition: 1 hour at room temperature
Counter stain: Hematoxylin
Comment: Color brown is the positive signal for bsm-60266R
Cell line: C2C12
Fixative: 4% Paraformaldehyde
Permeabilization: 0.1% TritonX-100
Primary ab dilution: 1:50
Primary incubation condition: 4°C overnight
Secondary ab: Goat Anti-Rabbit IgG
Nuclear counter stain: DAPI (Blue)
Comment: Color green is the positive signal for bsm-60266R
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