ALG9 Antibody, FITC conjugated

說明書

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) ALG9 Polyclonal antibody
Uniprot No.：

Q9H6U8
基因名：

ALG9
別名：

ALG9 antibody; ALG9_HUMAN antibody; Alpha-1,2-mannosyltransferase ALG9 antibody; Asparagine-linked glycosylation protein 9 homolog antibody; Disrupted in bipolar disorder protein 1 antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Alpha-1,2-mannosyltransferase ALG9 protein (434-618AA)
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：

>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.

功能：

Catalyzes the transfer of mannose from Dol-P-Man to lipid-linked oligosaccharides.
基因功能參考文獻：
1. ALG9 is upregulated in peripheral blood mononuclear cells of galactosaemia patients. PMID: 26733289
2. Our study shows that some pathogenic variants in ALG9 can present as a lethal skeletal dysplasia with visceral malformations as the most severe phenotype PMID: 25966638
3. Due to the ALG9 deficiency, cells accumulated the lipid-linked oligosaccharides Man(6)GlcNAc(2)-PP-dolichol and Man(8)GlcNAc(2)-PP-dolichol. PMID: 19451548
相關疾病：

Congenital disorder of glycosylation 1L (CDG1L); Gillessen-Kaesbach-Nishimura syndrome (GIKANIS)
亞細胞定位：

Endoplasmic reticulum membrane; Multi-pass membrane protein.
蛋白家族：

Glycosyltransferase 22 family
組織特異性：

Ubiquitously expressed; with highest levels in heart, liver and pancreas.
數據庫鏈接：

HGNC: 15672

OMIM: 263210

KEGG: hsa:79796

STRING: 9606.ENSP00000435517

UniGene: Hs.745155

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