Overview

  • Product nameAnti-BSCL2 antibody
    See all BSCL2 primary antibodies
  • Description
    Rabbit polyclonal to BSCL2
  • Tested applicationsSuitable for: WB, IHC-Pmore details
  • Species reactivity
    Reacts with: Mouse, Rat, Human
    Predicted to work with: Cow, Dog, Pig, Chimpanzee, Macaque Monkey, Gorilla, Orangutan
  • Immunogen

    Synthetic peptide conjugated to KLH derived from within residues 50 - 150 of Human BSCL2.

  • Positive control
    • This antibody gave a postive signal in the following tissue lysates: Human Brain; Mouse Brain; Rat Brain; Mouse Testis; Rat Testis. This antibody gave a positive result in IHC in the following FFPE tissue: Human normal testis.

Properties

Applications

Our Abpromise guarantee covers the use of ab106793 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB Use a concentration of 1 µg/ml. Detects a band of approximately 46 kDa (predicted molecular weight: 44 kDa).
IHC-P Use a concentration of 5 µg/ml.

Target

  • Tissue specificityHighest expression in brain and testis.
  • Involvement in diseaseDefects 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.
  • Sequence similaritiesBelongs to the seipin family.
  • Cellular localizationEndoplasmic reticulum membrane.
  • Information by UniProt
  • Database links
  • Alternative names
    • Bernardinelli Seip congenital lipodystrophy 2 antibody
    • Bernardinelli Seip congenital lipodystrophy type 2 protein antibody
    • Bernardinelli-Seip congenital lipodystrophy type 2 protein antibody
    • BSCL 2 antibody
    • BSCL2 antibody
    • BSCL2_HUMAN antibody
    • GNG3LG antibody
    • HMN 5 antibody
    • HMN5 antibody
    • MGC4694 antibody
    • Seipin antibody
    • Spastic paraplegia 17 (autosomal dominant) antibody
    • Spastic paraplegia 17 (Silver syndrome) antibody
    • Spastic paraplegia 17 antibody
    • Spastic paraplegia with amyotrophy of hands and feet (Silver syndrome) antibody
    • Spastic paraplegia with amyotrophy of hands and feet antibody
    • SPG 17 antibody
    • SPG17 antibody
    see all

Anti-BSCL2 antibody images

  • 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
    Goat Anti-Rabbit IgG H&L (HRP) preadsorbed (ab97080) at 1/5000 dilution
    Developed using the ECL technique

    Performed under reducing conditions.

    Predicted band size : 44 kDa
    Observed band size : 46 kDa (why is the actual band size different from the predicted?)
    Additional bands at : 100 kDa,75 kDa. We are unsure as to the identity of these extra bands.

    Exposure time : 3 minutes
    BSCL2 contains a number of potential glycosylation sites (SwissProt) which may explain its migration at a higher molecular weight than predicted.
  • All lanes : Anti-BSCL2 antibody (ab106793) at 1/1000 dilution

    Lane 1 : Wild type mouse white adipose tissue lysate (whole)
    Lane 2 : Knockout mouse white adipose tissue lysate (whole)

    Lysates/proteins at 50 µg per lane.

    Secondary
    HRP-conjugated goat anti-rabbit IgG polyclonal
    Developed using the ECL technique

    Performed under reducing conditions.

    Predicted band size : 44 kDa
    Observed band size : 48 kDa (why is the actual band size different from the predicted?)


    Exposure time : 5 seconds

    This image is courtesy of an Abreview submitted by Xavier Prieur

    See Abreview

  • 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.

References for Anti-BSCL2 antibody (ab106793)

This product has been referenced in:
  • Kociucka B  et al. Expression of genes involved in lipid droplet formation (BSCL2, SNAP23 and COPA) during porcine in vitro adipogenesis. J Appl Genet 57:505-510 (2016). Read more (PubMed: 27108337) »

See 1 Publication for this product

Product Wall

Application Western blot
Loading amount 50 µg
Gel Running Conditions Reduced Denaturing (4-15%)
Sample Mouse Tissue lysate - whole (white adipose tissue)
Specification white adipose tissue
Treatment WT versus KO
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 22°C
Username

Mr. Xavier Prieur

Verified customer

Submitted Jul 28 2014

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"