SGCA Antibody, FITC conjugated

說明書

中文名稱：

SGCA兔多克隆抗體, FITC偶聯
貨號：

CSB-PA623086LC01HU
規格：

￥880
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) SGCA Polyclonal antibody
Uniprot No.：

Q16586
基因名：

SGCA
別名：

50 DAG antibody; 50 kDa dystrophin associated glycoprotein antibody; 50 kDa dystrophin-associated glycoprotein antibody; 50DAG antibody; 50kD DAG antibody; 59kDa antibody; A2 antibody; adhalin antibody; ADL antibody; Alpha SG antibody; Alpha-sarcoglycan antibody; Alpha-SG antibody; Asg antibody; DAG2 antibody; DMDA2 antibody; Dystroglycan 2 antibody; Dystroglycan-2 antibody; LGMD2D antibody; sarcoglycan, alpha (dystrophin-associated glycoprotein) antibody; SCARMD1 antibody; Sgca antibody; SGCA_HUMAN antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Alpha-sarcoglycan protein (24-290AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

FITC
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

Antigen Affinity Purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
產品提供形式：

Liquid
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

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.
基因功能參考文獻：
1. Pathogenic mutations were found in SGCA from Egyptian families with limb-girdle muscular dystrophy. PMID: 26934379
2. IN TMD patients, a locus near the sarcoglycan alpha ( SGCA), rs4794106, was suggestive in the discovery analysis ( P = 2.6 x 10(6)) and replicated (i.e., 1-tailed P = 0.016) in the Brazilian cohort. PMID: 28081371
3. The results suggest that the carrier rate of c.101G>T in SGCA may be high in Taiwan, especially in the aboriginal population regardless of the tribes. PMID: 26944168
4. B4GAT1 is involved in the initiation of the LARGE-dependent repeating disaccharide that is necessary for extracellular matrix protein binding to O-mannosylated alpha-dystroglycan. PMID: 25279697
5. Results show that HRD1 and RFP2 contributes are required for the disposal of V247M alpha-sarcoglycan mutant. PMID: 24565866
6. 2 members of a Spanish family with muscular dystrophy had a new missense mutation c409G>A, p.Glu137Lys in exon 5 of the alpha-sarcoglycan gene, as well as a paternal c739G>A, p.Val24Met mutation inexon 6. PMID: 23703062
7. DNA analysis demonstrated homozygosity for a point mutation (574C>T) in the alpha-sarcoglycan gene. PMID: 22303798
8. E-cadherin,alpha-dystroglycan and beta-dystroglycan levels were decreased in the oesophageal primary tumour samples, despite the presence of normal levels of dystroglycan mRNA. PMID: 21884196
9. This study reported recessive founder LGMD2D for the Magdalen Islands, an archipelago settled in the XIXth century, largely by Acadian immigrants. PMID: 21856579
10. Reduced expression of laminin-binding glycans on alpha-DG may contribute to formation of highly infiltrative behavior of prostate carcinoma cells. PMID: 21656825
11. Peptide sequences in alpha-DG are substrates for protein-O-mannose N-acetylglucosaminyltransferase 1 (POMGnT1), demonstrated when a library of mannopeptides is generated which corresponds to sequences of the mucin-like stem region of alpha-DG. PMID: 21361872
12. Long-term, sustainable gene expression of alpha-sarcoglycan was observed following gene transfer mediated by AAV. PMID: 21031578
13. Absence of members of the dystrophin-associated glycoprotein complex constitutes a permissive environment for spontaneous development of embryonal rhabdomyosarcoma associated with mutation of p53 and mutation or altered splicing of Mdm2. PMID: 20019182
14. Two adult brothers with a mild form of LGMD2D, compound heterozygous for two missense mutations of the SGCA gene (Arg77Cys, Val247Met), presented with respiratory insufficiency while they were still ambulatory. PMID: 15298081
15. Biglycan is a ligand for two members of the sarcoglycan complex and regulates their expression at discrete developmental ages. PMID: 16883602
16. identified a negative regulatory element in the alpha-SG distal promoter including two conserved E-boxes (E1 and E2), which interact with MyoD PMID: 18078839
17. The limb-girdle muscular dystrophy patients with alpha-sarcoglycan deficient LGMD2D do not enable an accurate prediction of the genotype. PMID: 18996010
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相關疾?。?/div>
Limb-girdle muscular dystrophy 2D (LGMD2D)
亞細胞定位：

Cell membrane, sarcolemma; Single-pass type I membrane protein. Cytoplasm, cytoskeleton.
蛋白家族：

Sarcoglycan alpha/epsilon family
組織特異性：

Most strongly expressed in skeletal muscle. Also expressed in cardiac muscle and, at much lower levels, in lung. In the fetus, most abundant in cardiac muscle and, at lower levels, in lung. Also detected in liver and kidney. Not expressed in brain.
數據庫鏈接：

HGNC: 10805

OMIM: 600119

KEGG: hsa:6442

STRING: 9606.ENSP00000262018

UniGene: Hs.463412

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