DARS2 Antibody

中文名稱：

DARS2兔多克隆抗體
貨號：

CSB-PA747490LA01HU
規格：

￥440
圖片：
Western blot
All lanes: DARS2 antibody at 10μg/ml + MCF-7 whole cell lysate
Secondary
Goat polyclonal to rabbit IgG at 1/10000 dilution
Predicted band size: 74 kDa
Observed band size: 74 kDa

Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA747490LA01HU at dilution of 1:100
其他：

產品詳情
產品評價
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靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) DARS2 Polyclonal antibody
Uniprot No.：

Q6PI48
基因名：

DARS2
別名：

DARS2 antibody; Aspartate--tRNA ligase antibody; mitochondrial antibody; EC 6.1.1.12 antibody; Aspartyl-tRNA synthetase antibody; AspRS antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Aspartate--tRNA ligase, mitochondrial protein (489-628AA)
免疫原種屬：

Homo sapiens (Human)

標記方式：

Non-conjugated

本頁面中的產品，DARS2 Antibody (CSB-PA747490LA01HU)，的標記方式是Non-conjugated。對于DARS2 Antibody，我們還提供其他標記。見下表：

標記方式	貨號	產品名稱	應用
HRP	CSB-PA747490LB01HU	DARS2 Antibody, HRP conjugated	ELISA
FITC	CSB-PA747490LC01HU	DARS2 Antibody, FITC conjugated
Biotin	CSB-PA747490LD01HU	DARS2 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
產品提供形式：

Liquid
應用范圍：

ELISA, WB, IHC

推薦稀釋比：

Application	Recommended Dilution
WB	1:1000-1:5000
IHC	1:20-1:200

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

基因功能參考文獻：
1. Whole exome sequencing of the proband identified two compound heterozygous variants (NM_018122.4:c.1762C > G and c.563G > A) in DARS2 PMID: 30352563
2. Results show that DARS2 is strongly upregulated in hepatocellular carcinoma (HCC) and is associated with HCC progression. DARS2 promotes HCC tumorigenesis by accelerating cell cycle progression and attenuating cell apoptosis. PMID: 29052520
3. Mutations with mild effects on solubility occur in patients as allelic combinations whereas those with strong effects on solubility or on aminoacylation are necessarily associated with a partially functional allele. PMID: 26620921
4. This study identified DARS2-associated leukoencephalopathy with hypomyelination with brainstem and spinal cord involvement and leg spasticity. PMID: 25527264
5. 60 different DARS2 mutations were identified in 78 patients with leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation, 13 of which have not been reported before PMID: 24566671
6. Cognitive impairment seems to be common among patients with leukoencephalopathy with brainstem and spinal cord involvement and elevated lactate and DARS2 mutations PMID: 23652419
7. A comparison of biophysical properties of human mitochondrial aspartyl-tRNA synthetase, HsaDRS2, with them to those of a bacterial (E. coli) homolog, EcoDRS. PMID: 23275545
8. Pathogenic mutations causing LBSL affect mitochondrial aspartyl-tRNA synthetase in diverse way PMID: 23216004
9. We describe two new cases of Leukoencephalopathy with brainstem and spinal cord involvement and elevated lactate with a novel pathogenic mutation in the DARS2 gene PMID: 21792730
10. Case demonstrates that DARS2 mutation homozygosity is not lethal, as suggested earlier, but compatible with a rather benign disease course. PMID: 21749991
11. Leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation is associated with cell-type-dependent splicing of mtAspRS mRNA. PMID: 22023289
12. A novel homozygous mutation of DARS2 may cause a severe LBSL (Leukoencephalopathy with brain stem and spinal cord involvement with lactate elevation) variant. PMID: 21815884
13. This report describes two novel heterozygote composite mutations in the DARS2 gene PMID: 20878420
14. DARS2 mutations cause childhood-to-adolescence onset leucoencephalopathy, but they do not seem to be associated with multiple sclerosis. PMID: 19592391
15. The gene for mitochondrial aspartyl-tRNA synthetase is described and the initial characterization of the enzyme is reported. Genes for the remaining missing human synthetases have also been found with the exception of glutaminyl-tRNA synthetase. PMID: 15779907
16. Mutations in DARS2, which encodes mitochondrial aspartyl-tRNA synthetase, in affected individuals with leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation. PMID: 17384640
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相關疾病：

Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL)
亞細胞定位：

Mitochondrion matrix.
蛋白家族：

Class-II aminoacyl-tRNA synthetase family, Type 1 subfamily
數據庫鏈接：

HGNC: 25538

OMIM: 610956

KEGG: hsa:55157

STRING: 9606.ENSP00000355086

UniGene: Hs.647707

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