WB: Human, mouse and rat brain; Mouse and rat testis lysate.
IHC-P: Human testis tissue.
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
pH: 7.40 Preservative: 0.02% Sodium azide Constituent: PBS Note: Batches of this product that have a concentration < 1mg/ml may have BSA added as a stabilising agent. If you would like information about the formulation of a specific lot, please contact our scientific support team who will be happy to help.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 1 µg/ml. Detects a band of approximately 46 kDa (predicted molecular weight: 44 kDa).
Use a concentration of 5 µg/ml.
Highest expression in brain and testis.
Involvement in disease
Defects in BSCL2 are the cause of congenital generalized lipodystrophy type 2 (CGL2) [MIM:269700]. 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) [MIM:270685]; 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) [MIM:600794]; 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.
Spastic paraplegia with amyotrophy of hands and feet (Silver syndrome) antibody
Spastic paraplegia with amyotrophy of hands and feet antibody
SPG 17 antibody
Western blot - Anti-BSCL2 antibody (ab106793)
All lanes : Anti-BSCL2 antibody (ab106793) at 1 µg/ml
Lane 1 : Human brain tissue lysate - total protein (ab29466) Lane 2 : Brain (Mouse) Tissue Lysate Lane 3 : Brain (Rat) Tissue Lysate Lane 4 : Testis (Mouse) Tissue Lysate Lane 5 : Testis (Rat) Tissue Lysate
Lysates/proteins at 10 µg per lane.
Secondary All lanes : Goat Anti-Rabbit IgG H&L (HRP) preadsorbed (ab97080) at 1/5000 dilution
IHC image of BSCL2 staining in human normal testis formalin fixed paraffin embedded tissue section, performed on a Leica BondTM system using the standard protocol F. The section was pre-treated using heat mediated antigen retrieval with EDTA (pH9, epitope retrieval solution 2) for 20 mins. The section was then incubated with ab106793, 5µg/ml, for 15 mins at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.
For other IHC staining systems (automated and non-automated) customers should optimize variable parameters such as antigen retrieval conditions, primary antibody concentration and antibody incubation times.
Wang L et al. Seipin deficiency in mice causes loss of dopaminergic neurons via aggregation and phosphorylation of a-synuclein and neuroinflammation. Cell Death Dis9:440 (2018).
Read more (PubMed: 29670081) »
Mcilroy GD et al. Adipose specific disruption of seipin causes early-onset generalised lipodystrophy and altered fuel utilisation without severe metabolic disease. Mol Metab10:55-65 (2018).
Read more (PubMed: 29459250) »