B4GALT7 Antibody
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中文名稱：B4GALT7兔多克隆抗體
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貨號：CSB-PA890666LA01HU
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規格：￥440
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圖片：
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Western Blot
Positive WB detected in: Mouse brain tissue
All lanes: B4GALT7 antibody at 3μg/ml
Secondary
Goat polyclonal to rabbit IgG at 1/50000 dilution
Predicted band size: 38 kDa
Observed band size: 38 kDa
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其他：
產品詳情
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產品名稱：Rabbit anti-Homo sapiens (Human) B4GALT7 Polyclonal antibody
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Uniprot No.：
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基因名：B4GALT7
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別名：B4GALT7 antibody; XGALT1 antibody; UNQ748/PRO1478Beta-1,4-galactosyltransferase 7 antibody; Beta-1,4-GalTase 7 antibody; Beta4Gal-T7 antibody; b4Gal-T7 antibody; EC 2.4.1.- antibody; Proteoglycan UDP-galactose:beta-xylose beta1,4-galactosyltransferase I antibody; UDP-Gal:beta-GlcNAc beta-1,4-galactosyltransferase 7 antibody; UDP-galactose:beta-N-acetylglucosamine beta-1,4-galactosyltransferase 7 antibody; UDP-galactose:beta-xylose beta-1,4-galactosyltransferase antibody; XGPT antibody; XGalT-1 antibody; Xylosylprotein 4-beta-galactosyltransferase antibody; EC 2.4.1.133 antibody; Xylosylprotein beta-1,4-galactosyltransferase antibody
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宿主：Rabbit
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反應種屬：Human, Mouse
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免疫原：Recombinant Human Beta-1,4-galactosyltransferase 7 protein (52-327AA)
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免疫原種屬：Homo sapiens (Human)
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標記方式：Non-conjugated
本頁面中的產品，B4GALT7 Antibody (CSB-PA890666LA01HU)，的標記方式是Non-conjugated。對于B4GALT7 Antibody，我們還提供其他標記。見下表：
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克隆類型：Polyclonal
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抗體亞型：IgG
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純化方式：>95%, Protein G purified
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濃度：It differs from different batches. Please contact us to confirm it.
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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
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應用范圍：ELISA, WB
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推薦稀釋比：
Application Recommended Dilution WB 1:200-1:2000 -
Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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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.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
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功能：Required for the biosynthesis of the tetrasaccharide linkage region of proteoglycans, especially for small proteoglycans in skin fibroblasts.
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基因功能參考文獻：
- The phenotypes described in this article caused by bi-allelic mutations in B4GALT7 would benefit from reclassification and loss of its current association with PEDS. These conditions would be better grouped with the other linkeropathies. PMID: 26940150
- Our findings demonstrate that B4GALT7 is the causative gene for LRS. The identification of a unique homozygous mutation argues in favor of a founder effect. B4GALT7 encodes a galactosyltransferase. PMID: 24755949
- identified two key structural features forming stacking interactions with the aglycone, and the hydrogen bond between the His(195) nitrogen backbone and the carbonyl group of the coumarinyl molecule to develop a tight binder of hbeta4GalT7 PMID: 25568325
- a Michaelis complex of a glycosyltransferase has been described, and it clearly suggests an SN2 type catalytic mechanism for the beta4GalT7 enzyme. PMID: 24052259
- Two evolutionary conserved motifs, (163)DVD(165) and (221)FWGWGREDDE(230), are central in the organization of the enzyme active site. PMID: 20843813
- Mutated ennzyme affects glycosaminoglycan synthesis and is involved Ehlers-Danlos syndrome. PMID: 20809901
- This study establishes the molecular basis for beta4GalT7 defects associated with altered GAG synthesis in Ehlers-Danlos syndrome. PMID: 20691685
- phosphorylation of Xyl on the C-2 position prevents GalT-I activity PMID: 15522873
- reduced beta4GalT-7 activity resulting in defective glycosylation of decorin and biglycan may be responsible for the complex molecular pathology in beta4GalT-7 deficient Ehlers-Danlos syndrome patients PMID: 16583246
- Study suggests an heparan sulfate-dependent basic mechanism behind the altered wound repair phenotype of beta4GalT-7-deficient Ehlers-Danlos syndrome patients. PMID: 18158310
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相關疾病：Ehlers-Danlos syndrome with short stature and limb anomalies (EDSSLA)
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亞細胞定位：Golgi apparatus, Golgi stack membrane; Single-pass type II membrane protein. Note=Cis cisternae of Golgi stack.
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蛋白家族：Glycosyltransferase 7 family
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組織特異性：High expression in heart, pancreas and liver, medium in placenta and kidney, low in brain, skeletal muscle and lung.
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數據庫鏈接：
