产品详情
简单介绍：
Anti-BSCL2抗体产品质量稳定，实验效果明显，货期快，价格优惠，欢迎垂询订购！我公司长期供应**组化抗体、WB抗体、**组化试剂盒和抗体试验所需全部相关试剂、荧光标记抗体、单克隆抗体、多克隆抗体、各种标记的二抗IgG/IgM/IgD/IgA等科研实验抗体。Anti-BSCL2抗体用于**组化实验,WB实验,相应的标记抗体有HRP标记抗体,FITC标记,BIO等。
详情介绍：
Rabbit Anti-BSCL2
Cat. Number:
Anti-BSCL2抗体KL-6311R
Quantity size:
0.2ml
Concentration:
1mg/ml Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Background:
Defects in BSCL2 are the
Anti-BSCL2抗体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. Anti-BSCL2抗体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 Anti-BSCL2抗体of motor nerve fibers, predominantly in limb distal regions.
Defects in BSCL2 are the cause of spastic paraplegia type 17 (SPG17) ; also known as Silver spastic paraplegia syndrome. Anti-BSCL2抗体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 Anti-BSCL2抗体of motor nerve fibers, predominantly in limb distal regions.
Also known as:
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
Anti-BSCL2抗体feet (Silver syndrome); Spastic paraplegia with amyotrophy of hands and feet; SPG 17; SPG17.
Specificity:
●
Rabbit Polyclonal IgG, affinity purified by Protein A.
●
Reacts with: Human, Mouse, Rat, Dog, Cow, Horse, Rabbit, .
●
Immunogen: KLH conjugated synthetic peptide derived from human BSCL2/SPG17.
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Predicted Molecular Weight: 44kDa.
Storage:
Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).
Application:
WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500
Not yet tested in other applications.
Optimal working dilutions must be determined by the end user.
Not yet tested in other applications.
Optimal working dilutions must be determined by the end user.
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