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Product Name SGCE Chinese Name SGCE蛋白抗体 Alias dystonia 11, myoclonic; DYT11; Epsilon sarcoglycan; Epsilon SG; Epsilon-sarcoglycan; Epsilon-SG; ESG; sarcoglycan, epsilon; sgcE; SGCE_HUMAN. Research Area Cell biology Developmental biology Signal transduction Cytoskeleton Immunogen Species Rabbit Clonality Polyclonal React Species Mouse, Rat, (predicted: Human, Dog, Pig, Cow, Horse, Rabbit, Sheep, ) Applications WB=1:500-2000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 50kDa Cellular localization The cell membrane Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human SGCE: 61-160/437 <Extracellular> Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail This gene encodes the epsilon member of the sarcoglycan family. Sarcoglycans are transmembrane proteins that are components of the dystrophin-glycoprotein complex, which link the actin cytoskeleton to the extracellular matrix. Unlike other family members which are predominantly expressed in striated muscle, the epsilon sarcoglycan is more broadly expressed. Mutations in this gene are associated with myoclonus-dystonia syndrome. This gene is imprinted, with preferential expression from the paternal allele. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Oct 2010]
Function:
Component of the sarcoglycan complex, a subcomplex of the dystrophin-glycoprotein complex which forms a link between the F-actin cytoskeleton and the extracellular matrix.
Subcellular Location:
Cell membrane > sarcolemma. Cytoplasm > cytoskeleton.
Tissue Specificity:
Ubiquitous.
DISEASE:
Defects in SGCE are a cause of dystonia type 11 (DYT11) [MIM:159900]; also known as myoclonic dystonia or alcohol-responsive dystonia. DYT11 is a myoclonic dystonia. Dystonia is defined by the presence of sustained involuntary muscle contractions, often leading to abnormal postures. DYT11 is characterized by involuntary lightning jerks and dystonic movements and postures alleviated by alcohol. Inheritance is autosomal dominant. The age of onset, pattern of body involvement, presence of myoclonus and response to alcohol are all variable.
Similarity:
Belongs to the sarcoglycan alpha/epsilon family.
SWISS:
O43556
Gene ID:
8910
Database links:
Entrez Gene: 8910 Human
Entrez Gene: 20392 Mouse
Omim: 604149 Human
SwissProt: O43556 Human
SwissProt: O70258 Mouse
Unigene: 371199 Human
Unigene: 8739 Mouse
Unigene: 185815 Rat
Product Picture Sample:
Lung (Mouse) Lysate at 40 ug
Kidney (Mouse) Lysate at 40 ug
Primary: Anti-SGCE (SL17311R) at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 50 kD
Observed band size: 50 kD
Paraformaldehyde-fixed, paraffin embedded (Mouse brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (SGCE) Polyclonal Antibody, Unconjugated (SL17311R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.Paraformaldehyde-fixed, paraffin embedded (Rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (SGCE) Polyclonal Antibody, Unconjugated (SL17311R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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