B4GALT7 Antibody

說(shuō)明書(shū)

中文名稱：

B4GALT7兔多克隆抗體
貨號(hào)：

CSB-PA890666LA01HU
規(guī)格：

￥440
圖片：
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
其他：

產(chǎn)品詳情
產(chǎn)品評(píng)價(jià)
相關(guān)產(chǎn)品
靶點(diǎn)詳情

產(chǎn)品詳情

產(chǎn)品名稱：

Rabbit anti-Homo sapiens (Human) B4GALT7 Polyclonal antibody
Uniprot No.：

Q9UBV7
基因名：

B4GALT7
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
宿主：

Rabbit
反應(yīng)種屬：

Human, Mouse
免疫原：

Recombinant Human Beta-1,4-galactosyltransferase 7 protein (52-327AA)
免疫原種屬：

Homo sapiens (Human)

標(biāo)記方式：

Non-conjugated

本頁(yè)面中的產(chǎn)品，B4GALT7 Antibody (CSB-PA890666LA01HU)，的標(biāo)記方式是Non-conjugated。對(duì)于B4GALT7 Antibody，我們還提供其他標(biāo)記。見(jiàn)下表：

標(biāo)記方式	貨號(hào)	產(chǎn)品名稱	應(yīng)用
HRP	CSB-PA890666LB01HU	B4GALT7 Antibody, HRP conjugated	ELISA
FITC	CSB-PA890666LC01HU	B4GALT7 Antibody, FITC conjugated
Biotin	CSB-PA890666LD01HU	B4GALT7 Antibody, Biotin conjugated	ELISA

克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G 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(chǎn)品提供形式：

Liquid
應(yīng)用范圍：

ELISA, WB

推薦稀釋比：

Application	Recommended Dilution
WB	1:200-1:2000

Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
儲(chǔ)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(chǎn)品評(píng)價(jià)

靶點(diǎn)詳情

功能：

Required for the biosynthesis of the tetrasaccharide linkage region of proteoglycans, especially for small proteoglycans in skin fibroblasts.
基因功能參考文獻(xiàn)：
1. 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
2. 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
3. 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
4. a Michaelis complex of a glycosyltransferase has been described, and it clearly suggests an SN2 type catalytic mechanism for the beta4GalT7 enzyme. PMID: 24052259
5. Two evolutionary conserved motifs, (163)DVD(165) and (221)FWGWGREDDE(230), are central in the organization of the enzyme active site. PMID: 20843813
6. Mutated ennzyme affects glycosaminoglycan synthesis and is involved Ehlers-Danlos syndrome. PMID: 20809901
7. This study establishes the molecular basis for beta4GalT7 defects associated with altered GAG synthesis in Ehlers-Danlos syndrome. PMID: 20691685
8. phosphorylation of Xyl on the C-2 position prevents GalT-I activity PMID: 15522873
9. 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
10. 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(guān)疾病：

Ehlers-Danlos syndrome with short stature and limb anomalies (EDSSLA)
亞細(xì)胞定位：

Golgi apparatus, Golgi stack membrane; Single-pass type II membrane protein. Note=Cis cisternae of Golgi stack.
蛋白家族：

Glycosyltransferase 7 family
組織特異性：

High expression in heart, pancreas and liver, medium in placenta and kidney, low in brain, skeletal muscle and lung.
數(shù)據(jù)庫(kù)鏈接：

HGNC: 930

OMIM: 130070

KEGG: hsa:11285

STRING: 9606.ENSP00000029410

UniGene: Hs.455109

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