Desmin结蛋白抗体_详细资料

产品信息

保存条件： Store at -20 °C 浓度： 1mg/ml 应用范围： WB=1:500-2000 Flow-Cyt=1μg/Test （石蜡切片需做抗原修复）形态： Lyophilized or Liquid 亚型： IgG 免疫原： KLH conjugated synthetic peptide derived from human Desmin:401-470/470 适应物种： Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, 宿主： Rabbit 抗体名：结蛋白抗体抗体英文名： Desmin 保质期： 1年抗原来源： Rabbit 克隆性：多克隆目录编号： LM-20701R 数量：大量供应商：上海联迈生物工程有限公司规格： 100ul  200ul

Desmin结蛋白抗体

英文名称 Desmin

中文名称结蛋白抗体

别    名 CMD1I; CSM1; CSM2; DES; FLJ12025; FLJ39719; FLJ41013; FLJ41793; Intermediate filament protein; OTTHUMP00000064865; DESM_HUMAN; Desmin; FLJ12025; FLJ39719; FLJ41013; FLJ41793.

规格价格 100ul/1380元购买    200ul/2200元购买    大包装/询价

说明书 100ul  200ul

研究领域肿瘤  细胞生物  免疫学  信号转导  细胞类型标志物

抗体来源 Rabbit

克隆类型 Polyclonal

交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,

产品应用 WB=1:500-2000 Flow-Cyt=1μg/Test （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.

分子量 52kDa

细胞定位细胞浆

性    状 Lyophilized or Liquid

浓    度 1mg/ml

免疫原 KLH conjugated synthetic peptide derived from human Desmin:401-470/470

亚    型 IgG

纯化方法 affinity purified by Protein A

储存液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

PubMed PubMed

产品介绍 background:
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. Defects in Desmin are the cause of desmin related cardio skeletal myopathy (CSM) also known as desmin related myopathy (DRM). CSM is characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and by intracytoplasmic accumulation of desmin reactive deposits in cardiac and skeletal muscle cells. A desmin related myopathy can have a distal onset, it is then known as hereditary distal myopathy (HDM). Defects in Desmin are also the cause of dilated cardiomyopathy type 1I (CMD1I). CMD1I is an autosomal form of dilated cardiomyopathy characterized by ventricular dilatation and impaired systolic function. Antidesmin antibodies are useful in identification of tumours of myogenic origin.

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

Omim: 125660 Human

SwissProt: P17661 Human

SwissProt: P31001 Mouse

SwissProt: P48675 Rat

Unigene: 594952 Human

Unigene: 6712 Mouse

Unigene: 39196 Rat

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

Desmin在很多哺乳动物中的横纹肌和各种平滑肌及其来源的肿瘤组织中都有表达。结蛋白是一种中间丝蛋白，广泛分布于骨骼肌细胞、平滑肌细胞、心肌细胞和肌上皮细胞及其肿瘤中，主要用于子宫、皮肤、胃肠道及其它横纹肌肉瘤和肌上皮瘤的诊断和鉴别诊断。

产品图片
Sample:
Muscle (Mouse) Lysate at 40 ug
Stomach (Mouse) Lysate at 40 ug
Heart (Mouse) Lysate at 40 ug
Primary: Anti- Desmin (bs-20701R)at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 52kD
Observed band size: 52kD

Sample:
Heart (Mouse) Lysate at 40 ug
Muscle (Mouse) Lysate at 40 ug
Primary: Anti- Desmin (bs-20701R) at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 52 kD
Observed band size: 52 kD

Blank control (black line): A431(black) (The cells were fixed with 2% paraformaldehyde (10 min) , then permeabilized with PBST for 30 min on room temperature)
Primary Antibody (green line): Rabbit Anti-Desmin antibody (bs-20701R) ; Dilution: 1μg /10^6 cells;
Isotype Control Antibody (orange line): Rabbit IgG .
Secondary Antibody (white blue line): Goat anti-rabbit IgG-FITC;Dilution: 1μg /test.

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英文名称	Desmin
中文名称	结蛋白抗体
别名	CMD1I; CSM1; CSM2; DES; FLJ12025; FLJ39719; FLJ41013; FLJ41793; Intermediate filament protein; OTTHUMP00000064865; DESM_HUMAN; Desmin; FLJ12025; FLJ39719; FLJ41013; FLJ41793.
规格价格	100ul/1380元购买 200ul/2200元购买大包装/询价
说明书	100ul 200ul
研究领域	肿瘤细胞生物免疫学信号转导细胞类型标志物
抗体来源	Rabbit
克隆类型	Polyclonal
交叉反应	Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,
产品应用	WB=1:500-2000 Flow-Cyt=1μg/Test （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.
分子量	52kDa
细胞定位	细胞浆
性状	Lyophilized or Liquid
浓度	1mg/ml
免疫原	KLH conjugated synthetic peptide derived from human Desmin:401-470/470
亚型	IgG
纯化方法	affinity purified by Protein A
储存液	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件	Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
PubMed	PubMed
产品介绍	background: 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. Defects in Desmin are the cause of desmin related cardio skeletal myopathy (CSM) also known as desmin related myopathy (DRM). CSM is characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and by intracytoplasmic accumulation of desmin reactive deposits in cardiac and skeletal muscle cells. A desmin related myopathy can have a distal onset, it is then known as hereditary distal myopathy (HDM). Defects in Desmin are also the cause of dilated cardiomyopathy type 1I (CMD1I). CMD1I is an autosomal form of dilated cardiomyopathy characterized by ventricular dilatation and impaired systolic function. Antidesmin antibodies are useful in identification of tumours of myogenic origin. 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 Omim: 125660 Human SwissProt: P17661 Human SwissProt: P31001 Mouse SwissProt: P48675 Rat Unigene: 594952 Human Unigene: 6712 Mouse Unigene: 39196 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. Desmin在很多哺乳动物中的横纹肌和各种平滑肌及其来源的肿瘤组织中都有表达。结蛋白是一种中间丝蛋白，广泛分布于骨骼肌细胞、平滑肌细胞、心肌细胞和肌上皮细胞及其肿瘤中，主要用于子宫、皮肤、胃肠道及其它横纹肌肉瘤和肌上皮瘤的诊断和鉴别诊断。
产品图片	Sample: Muscle (Mouse) Lysate at 40 ug Stomach (Mouse) Lysate at 40 ug Heart (Mouse) Lysate at 40 ug Primary: Anti- Desmin (bs-20701R)at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 52kD Observed band size: 52kD Sample: Heart (Mouse) Lysate at 40 ug Muscle (Mouse) Lysate at 40 ug Primary: Anti- Desmin (bs-20701R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 52 kD Observed band size: 52 kD Blank control (black line): A431(black) (The cells were fixed with 2% paraformaldehyde (10 min) , then permeabilized with PBST for 30 min on room temperature) Primary Antibody (green line): Rabbit Anti-Desmin antibody (bs-20701R) ; Dilution: 1μg /10^6 cells; Isotype Control Antibody (orange line): Rabbit IgG . Secondary Antibody (white blue line): Goat anti-rabbit IgG-FITC;Dilution: 1μg /test.