EARS2 Antibody, FITC conjugated

說明書

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) EARS2 Polyclonal antibody
Uniprot No.：

Q5JPH6
基因名：

EARS2
別名：

3230401I01Rik antibody; AL024049 antibody; COXPD12 antibody; EARS 2 antibody; ears2 antibody; GluRS antibody; Glutamate--tRNA ligase antibody; Glutamyl tRNA synthetase 2 mitochondrial antibody; KIAA1970 antibody; mitochondrial antibody; mKIAA1970 antibody; MSE1 antibody; Probable glutamyl tRNA synthetase; mitochondrial antibody; Probable glutamyl-tRNA synthetase antibody; RGD1307904 antibody; SYEM_HUMAN antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Probable glutamate--tRNA ligase, mitochondrial protein (42-166AA)
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 attachment of glutamate to tRNA(Glu) in a two-step reaction: glutamate is first activated by ATP to form Glu-AMP and then transferred to the acceptor end of tRNA(Glu).
基因功能參考文獻：
1. This study presented that a new neurological disease, early-onset leukoencephalopathy with thalamus and brainstem involvement and high lactate, defined by a peculiar biphasic clinical course and caused by mutations in a single gene, EARS2. PMID: 22492562
相關疾病：

Combined oxidative phosphorylation deficiency 12 (COXPD12)
亞細胞定位：

Mitochondrion matrix.
蛋白家族：

Class-I aminoacyl-tRNA synthetase family, Glutamate--tRNA ligase type 1 subfamily
數據庫鏈接：

HGNC: 29419

OMIM: 612799

KEGG: hsa:124454

STRING: 9606.ENSP00000343488

UniGene: Hs.620541

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