AGA Antibody

說明書

中文名稱：

AGA兔多克隆抗體
貨號：

CSB-PA443866
規格：

￥1100
圖片：
The image on the left is immunohistochemistry of paraffin-embedded Human breast cancer tissue using CSB-PA443866(AGA Antibody) at dilution 1/20, on the right is treated with fusion protein. (Original magnification: ×200)

The image on the left is immunohistochemistry of paraffin-embedded Human lung cancer tissue using CSB-PA443866(AGA Antibody) at dilution 1/20, on the right is treated with fusion protein. (Original magnification: ×200)

Gel: 10%SDS-PAGE, Lysate: 40 μg, Lane 1-3: Mouse kidney tissue, human testis tissue, Human thyroid cancer tissue, Primary antibody: CSB-PA443866(AGA Antibody) at dilution 1/100, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 10 seconds
其他：

產品詳情
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靶點詳情

產品詳情

Uniprot No.：

P20933
基因名：

AGA
別名：

Aga antibody; AGU antibody; Aspartylglucosaminidase antibody; Aspartylglucosylamine deaspartylase antibody; Aspartylglycosaminuria antibody; ASPG_HUMAN antibody; ASRG antibody; GA antibody; Glycosylasparaginase antibody; Glycosylasparaginase beta chain antibody; N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase antibody; N4 (N acetyl beta glucosaminyl) L asparagine amidase antibody; N4-(N-acetyl-beta-glucosaminyl)-L-asparagine amidase antibody
宿主：

Rabbit
反應種屬：

Human,Mouse,Rat
免疫原：

Fusion protein of Human AGA
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
抗體亞型：

IgG
純化方式：

Antigen affinity purification
濃度：

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

-20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
產品提供形式：

Liquid
應用范圍：

ELISA,WB,IHC

推薦稀釋比：

Application	Recommended Dilution
ELISA	1:1000-1:2000
WB	1:200-1:1000
IHC	1:15-1:50

Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunohistochemistry (IHC) Protocol
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：

Cleaves the GlcNAc-Asn bond which joins oligosaccharides to the peptide of asparagine-linked glycoproteins.
基因功能參考文獻：
1. 1.8A resolution crystal structure of mature G172D mutant of a model missense GA corresponding to a Canadian aspartylglucosaminuria allele; studied the effect of its single amino acid change on substrate processing PMID: 28457719
2. We show that gene-silenced cells show specifically reduced AGA activity and store globotriaosylceramide. In gene-silenced cells, release of the neurotransmitter acetylcholine is significantly reduced, demonstrating that this model may be used to study specific neuronal functions such as neurotransmitter release in Fabry disease PMID: 27471012
3. study reports 2 novel aspartylglucosaminidase gene mutations, one in Qatari twins with an early, perinatal presentation not previously described for aspartylglucosaminuria and the other in 3 Turkish children with newly diagnosed aspartylglucosaminuria and a more classical disease course PMID: 23271757
4. [review] Natural killer (NK) cell tumors, subtypes of myeloid leukemias and T-cell lymphomas respond to ASNase; ovarian carcinomas and other solid tumors have been proposed as additional targets for ASNase, with a potential role for glutaminase. activity. PMID: 21854356
5. Molecular mechanism for the autoproteolytic activation of aspartylglucosaminidase. PMID: 14616088
6. A new point mutation, c.44T>G, found in a Finnish compound heterozygote causes a L15R AA substitution in the signal sequence of the AGA enzyme, affecting AGA translocation by altering a critical hydrophobic core structure in the signal sequence. PMID: 15365992
7. aspartylglucosaminidase may have a role in development of congenital disorders of glycosylation type I PMID: 16435229
8. The amino acid substitutions in aspartylglucosaminidase responsible for aspartylglucosaminuria were classified and divided in three groups. PMID: 18992224
9. Increased AGA plasma activity, although a consistent finding in congenital disorders of glycosylation patients, is not specific to this group of disorders. PMID: 19100247
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相關疾病：

Aspartylglucosaminuria (AGU)
亞細胞定位：

Lysosome.
蛋白家族：

Ntn-hydrolase family
數據庫鏈接：

HGNC: 318

OMIM: 208400

KEGG: hsa:175

STRING: 9606.ENSP00000264595

UniGene: Hs.207776

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