Berardinelli-Seip Congenital Lipodystrophy 2 (Seipin) Proteins (BSCL2)

BSCL2 encodes the multi-pass transmembrane protein protein seipin. Additionally we are shipping BSCL2 Antibodies (39) and BSCL2 Kits (4) and many more products for this protein.

list all proteins Gene Name GeneID UniProt
BSCL2 26580 Q96G97
Rat BSCL2 BSCL2 361722 Q5FVJ6
BSCL2 14705 Q9Z2E9
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Top BSCL2 Proteins at antibodies-online.com

Showing 6 out of 9 products:

Catalog No. Origin Source Conjugate Images Quantity Supplier Delivery Price Details
Insect Cells Mouse rho-1D4 tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 0.25 mg Log in to see 50 to 55 Days
$4,244.78
Details
HOST_Escherichia coli (E. coli) Mouse His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 30 to 35 Days
$4,331.68
Details
HOST_Escherichia coli (E. coli) Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 30 to 35 Days
$4,331.68
Details
Insect Cells Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 50 Days
$5,442.50
Details
HOST_HEK-293 Cells Human Myc-DYKDDDDK Tag Validation with Western Blot 20 μg Log in to see 10 to 12 Days
$785.40
Details
HOST_Wheat germ Human GST tag 10 μg Log in to see 11 to 12 Days
$405.71
Details

BSCL2 Proteins by Origin and Source

Origin Expressed in Conjugate
Human , , ,
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Mouse (Murine) ,
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More Proteins for Berardinelli-Seip Congenital Lipodystrophy 2 (Seipin) (BSCL2) Interaction Partners

Human Berardinelli-Seip Congenital Lipodystrophy 2 (Seipin) (BSCL2) interaction partners

  1. Results provide evidence that the hepatic BSCL2 deficiency induces the increase and expansion of lipid droplets potentially via increased SCD1 (show SCD Proteins) activity.

  2. results suggest that Celia seipin is probably playing an underestimated role in adipocyte maturation, but not in senescence, and its expression can be modified by exogenous factors as fatty acids.

  3. Data show that all three patients exhibited characteristic features of congenital generalized lipodystrophy (CGL (show MGAT1 Proteins)) due to mutations in the Bernardinelli-Seip congenital (BSCL2) gene.

  4. Together, these data suggest that seipin helps to connect newly formed lipid droplets to the endoplasmic reticulum and that by stabilizing endoplasmic reticulum-lipid droplet contacts seipin facilitates the incorporation of protein and lipid cargo into growing lipid droplets in human cells.

  5. Increased aggregation and subsequent impaired oligomerization of Celia seipin leads to cell death. In heterozygous carriers, wildtype seipin might prevent the damage caused by mutant seipin through its sequestration into harmless mixed oligomers.

  6. BSCL2 defines the localization of adipose differentiation-related protein (show PLIN2 Proteins), which has a role in lipid accumulation and adipogenic differentiation

  7. BSCL2 mutations account for a small number of patients with inherited neuropathies in Taiwan. The p.R96H mutation is associated with distal hereditary motor neuropathy.

  8. We confirmed reduction of brain volume and number of sperm in human patients with BSCL2 mutation. This is the first report demonstrating that seipin is necessary for normal brain development and spermatogenesis

  9. The S90L mutationof BSCL2 is predominantly associated with Silver syndrome

  10. The mutation of seipin at glycosylation sites disrupt its function in regulating lipid droplet metabolism, and the autophagy acts as an adaptive response to break down abnormal lipid droplets.

Mouse (Murine) Berardinelli-Seip Congenital Lipodystrophy 2 (Seipin) (BSCL2) interaction partners

  1. Findings reveal a key cell-autonomous role for BSCL2 in controlling brown adipose tissue mass and activity.

  2. Our findings reveal that seipin knockout exacerbates cerebral I/R-induced damages by increasing BBB permeability, amplifying ER stress and increasing glucose levels, as well as decreasing leptin (show LEP Proteins) and adiponectin (show ADIPOQ Proteins) levels, indicating that seipin may be a potential therapeutic target for stroke.

  3. The results indicate that, by reducing PPARgamma (show PPARG Proteins), seipin deficiency impairs proliferation and differentiation of neural stem and progenitor cells.

  4. Authors generated adipose tissue (mature) Bscl2 knockout (Ad-mKO) mice to investigate the impact of acquired Bscl2 deletion on adipose tissue function and energy balance.

  5. The present study provides in vivo evidence that neuronal seipin deficiency leads to spatial cognitive deficits thtat can be rescued by the activation of PPARgamma (show PPARG Proteins).

  6. spermatid apoptosis, increased chromocenter fragmentation, abnormal acrosome formation, and defective mitochondrial activity contributed to decreased sperm production and defective sperm that resulted in Bscl2-/- male infertility.

  7. Bscl2(-/-) females have accelerated postnatal mammary ductal development but delayed vaginal opening; they display segregated responses in mammary gland development and vaginal opening to prepubertal genistein treatment.

  8. Using the lipodystrophic Seipin-deficient mouse (Seipin(-/-)) model, the study found Seipin(-/-) mice were unable to respond appropriately to a long time fasting and developed postprandial hypertriglyceridemia.

  9. a brief overview of the genetic association of the CGLs, and focus on the current understanding of differential contributions of distinct seipin domains to lipid storage and adipogenesis.

  10. Neuronal seipin deficiency causing reduced PPARgamma (show PPARG Proteins) levels leads to affective disorders in male mice that are rescued by estradiol-increased PPARgamma (show PPARG Proteins) expression.

BSCL2 Protein Profile

Protein Summary

This gene encodes the multi-pass transmembrane protein protein seipin. This protein localizes to the endoplasmic reticulum and may be important for lipid droplet morphology. Mutations in this gene have been associated with congenital generalized lipodystrophy type 2 or Berardinelli-Seip syndrome, a rare autosomal recessive disease characterized by a near absence of adipose tissue and severe insulin resistance. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. Naturally occurring read-through transcription occurs between this locus and the neighboring locus HNRNPUL2 (heterogeneous nuclear ribonucleoprotein U-like 2).

Gene names and symbols associated with BSCL2

  • Berardinelli-Seip congenital lipodystrophy 2 (seipin) (BSCL2)
  • Berardinelli-Seip congenital lipodystrophy 2 (seipin) (Bscl2)
  • Bernardinelli-Seip congenital lipodystrophy 2 homolog (human) (Bscl2)
  • 2900097C17Rik protein
  • AI046355 protein
  • Gng3lg protein
  • HMN5 protein
  • SPG17 protein

Protein level used designations for BSCL2

Bernardinelli-Seip congenital lipodystrophy type 2 protein , seipin , Bernardinelli-Seip congenital lipodystrophy 2 (seipin) , bernardinelli-Seip congenital lipodystrophy type 2 protein homolog , G protein gamma 3 linked

GENE ID SPECIES
26580 Homo sapiens
476054 Canis lupus familiaris
513558 Bos taurus
361722 Rattus norvegicus
14705 Mus musculus
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