SGCE Antibody (PACO60821)
- SKU:
- PACO60821
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
抗体名: | SGCE Antibody (PACO60821) |
抗体コード: | PACO60821 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA, IHC |
推奨される希釈: | ELISA:1:2000-1:10000, IHC:1:200-1:500 |
反応性: | Human |
免疫原: | Recombinant Human Epsilon-sarcoglycan protein (8-126AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Non-conjugated |
![]() | IHC image of PACO60821 diluted at 1:300 and staining in paraffin-embedded human skeletal muscle tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system. |
バックグラウンド: | 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. |
シノニム: | Epsilon-sarcoglycan (Epsilon-SG), SGCE, ESG |
UniProt Protein Function: | SGCE: 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. Defects in SGCE are a cause of dystonia type 11 (DYT11); 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. Belongs to the sarcoglycan alpha/epsilon family. |
UniProt Protein Details: | Protein type:Membrane protein, integral; Cell adhesion Chromosomal Location of Human Ortholog: 7q21.3 Cellular Component: dystrophin-associated glycoprotein complex; Golgi apparatus; integral to plasma membrane; plasma membrane Biological Process: cell-matrix adhesion; muscle development; muscle system process Disease: Myoclonic Dystonia |
NCBI Summary: | 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] |
UniProt Code: | O43556 |
NCBI GenInfo Identifier: | 251757514 |
NCBI Gene ID: | 8910 |
NCBI Accession: | O43556.6 |
UniProt Secondary Accession: | O43556,Q6L8P0, Q75MH8, Q8NFG8, Q8WW28, B2R8N2, D6W5Q8 E9PF60, G5E9K6, |
UniProt Related Accession: | O43556 |
Molecular Weight: | 52,525 Da |
NCBI Full Name: | Epsilon-sarcoglycan |
NCBI Synonym Full Names: | sarcoglycan epsilon |
NCBI Official Symbol: | SGCE |
NCBI Official Synonym Symbols: | ESG; DYT11 |
NCBI Protein Information: | epsilon-sarcoglycan |
UniProt Protein Name: | Epsilon-sarcoglycan |
Protein Family: | Protein |
UniProt Gene Name: | SGCE |
UniProt Entry Name: | SGCE_HUMAN |