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Anti-BSCL2 Polyclonal Antibody is a Rabbit antibody targeting BSCL2. Anti-BSCL2 Polyclonal Antibody can be used in IHC-P,IHC-Fr,IF.
| Pack Size | Price | USA Warehouse | Global Warehouse | Quantity |
|---|---|---|---|---|
| 50 μL | $223 | 7-10 days | 7-10 days | |
| 100 μL | $373 | 7-10 days | 7-10 days | |
| 200 μL | $528 | 7-10 days | 7-10 days |
| Description | Anti-BSCL2 Polyclonal Antibody is a Rabbit antibody targeting BSCL2. Anti-BSCL2 Polyclonal Antibody can be used in IHC-P,IHC-Fr,IF. |
| Ig Type | IgG |
| Reactivity | Rat (predicted:Human,Mouse,Dog,Cow,Horse,Rabbit) |
| Verified Activity | Tissue/cell: rat brain tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer (0.01 M, pH 6.0), Boiling bathing for 15 min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30 min; Blocking buffer (normal goat serum) at 37℃ for 20 min; Incubation: Anti-BSCL2 Polyclonal Antibody, Unconjugated (TMAB-03234) 1: 200, overnight at 4°C, followed by conjugation to the secondary antibody and DAB staining |
| Application | |
| Recommended Dose | IHC-P: 1:100-500; IHC-Fr: 1:100-500; IF: 1:100-500 |
| Antibody Type | Polyclonal |
| Host Species | Rabbit |
| Tissue Specificity | Highest expression in brain and testis. |
| Construction | Polyclonal Antibody |
| Purification | Protein A purified |
| Appearance | Liquid |
| Formulation | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Concentration | 1mg/ml |
| Research Background | 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. |
| Immunogen | KLH conjugated synthetic peptide: human BSCL2/SPG17 |
| Antigen Species | Human |
| Gene Name | BSCL2 |
| Gene ID | |
| Protein Name | Seipin |
| Function | Endoplasmic reticulum membrane; Multi-pass membrane protein. |
| Molecular Weight | Theoretical: 44 kDa. |
| Stability & Storage | Store at 2°C-8°C for 1 month. Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles. |
| Transport | Shipping with blue ice. |
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