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| 规格: | 50ul | 产品价格: | ¥1180.0 |
|---|---|---|---|
| 规格: | 100ul | 产品价格: | ¥1980.0 |
| 规格: | 200ul | 产品价格: | ¥2800.0 |
| 产品编号 | bs-6311R |
| 英文名称 | BSCL2 Rabbit pAb |
| 中文名称 | 先天性脂肪代谢障碍蛋白2抗体(常染色体显性遗传痉挛性截瘫17) |
| 英文别名 | Bernardinelli Seip congenital lipodystrophy 2; Bernardinelli Seip congenital lipodystrophy type 2 protein; Bernardinelli-Seip congenital lipodystrophy type 2 protein; BSCL 2; BSCL2; BSCL2_HUMAN; GNG3LG; HMN 5; HMN5; MGC4694; Seipin; Spastic paraplegia 17(autosomal dominant); Spastic paraplegia 17(Silver syndrome); Spastic paraplegia 17; Spastic paraplegia with amyotrophy of hands and feet(Silver syndrome); Spastic paraplegia with amyotrophy of hands and feet; SPG 17; SPG17. |
| 产品应用 | IHC-P=1:100-500, IHC-F=1:100-500, IF=1:100-500 Not yet tested in other applications. |
| 交叉反应 | Rat (Human, Mouse, Dog, Cow, Horse, Rabbit) |
| 抗体来源 | Rabbit |
| 免疫原 | KLH conjugated synthetic peptide derived from human BSCL2/SPG17 |
| 亚型 | IgG |
| 性状 | Liquid |
| 纯化方法 | affinity purified by Protein A |
| 克隆类型 | Polyclonal |
| 理论分子量 | 44 kDa |
| 浓度 | 1mg/ml |
| 储存液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 研究领域 | Cardiovascular > Atherosclerosis > Diabetes associated Metabolism > Types of disease > Obesity Neuroscience > Neurology process > Neurodegenerative disease Tags & Cell Markers > Subcellular Markers > Organelles > ER |
| 组织特异性 | Highest expression in brain and testis. |
| 相似性 | Belongs to the seipin family. |
| 功能 | Endoplasmic reticulum membrane; Multi-pass membrane protein. |
| 保存条件 | Shipped at 4℃. Store at -20℃ 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. |
| 背景资料 | Defects in BSCL2 are the cause of congenital generalized lipodystrophy type 2 (CGL2) . Congenital generalized lipodystrophy is an autosomal recessive disorder characterized by a near absence of adipose tissue, extreme insulin resistance, hypertriglyceridemia, hepatic steatosis and early onset of diabetes.Defects in BSCL2 are the cause of spastic paraplegia type 17 (SPG17) ; also known as Silver spastic paraplegia syndrome. Spastic paraplegia is a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. SPG17 is characterized by prominent amyotrophy of the hand muscles, the presence of mild to severe pyramidal tract signs, and spastic paraplegia. SPG17 is a motor neuron disease overlapping with distal spinal muscular atrophy type 5.Defects in BSCL2 are a cause of distal hereditary motor neuropathy type 5 (HMN5); also known aS distal hereditary motor neuropathy type V (DSMAV). HMN5 is an autosomal dominant disorder characterized by degeneration of motor nerve fibers, predominantly in limb distal regions. |
| 应用 | 推荐稀释比例 |
| {IHC-P} | {1:100-500} |
| {IHC-F} | {1:100-500} |
| {IF} | {1:100-500} |

Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
Incubation: Anti-BSCL2 Polyclonal Antibody, Unconjugated(bs-6311R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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文献和实验= NAL), appears to form upon oxidative cyclization of the nonfluorescent 2:1 lysine-HNE Michael adduct-Schiff base cross-link (Scheme 1). Polyclonal antibody (PAb) to the NAL-HNE fluorophore was raised in rabbit and found to be highly specific
GE Healthcare Benzamidine Sepharose™ 6B is p-aminobenzamidine covalently attached to Sepharose 6B by the epoxy coupling method. p-Aminobenzamidine (PAB), is a synthetic inhibitor of trypsin-like serine protease. Trypsin and trypsin
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.g., Sigma) 50 mg/ml bis‐sulfosuccinimdylsuberate (BS3 ;Pierce, cat. no. PI 21580) in dimethylsulfoxide (DMSO) 5 mg/ml
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