ERCC6L2 Antibody

中文名稱：

ERCC6L2兔多克隆抗體
貨號：

CSB-PA004261LA01HU
規格：

￥440
圖片：
Immunohistochemistry of paraffin-embedded human lung tissue using CSB-PA004261LA01HU at dilution of 1:100
其他：

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) ERCC6L2 Polyclonal antibody
Uniprot No.：

Q5T890
基因名：

ERCC6L2
別名：

C9orf102 antibody; Chromosome 9 open reading frame 102 antibody; excision repair cross-complementing rodent repair deficiency; complementation group 6-like 2 antibody; FLJ37706 antibody; MGC30192 antibody; MGC43364 antibody; Putative DNA repair and recombination protein RAD26-like antibody; putative repair and recombination helicase RAD26L antibody; RAD26_HUMAN antibody; Rad26l antibody; RAD26L hypothetical protein antibody; SR278 antibody; stretch responsive protein 278 antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human DNA excision repair protein ERCC-6-like 2 protein (72-371AA)
免疫原種屬：

Homo sapiens (Human)

標記方式：

Non-conjugated

本頁面中的產品，ERCC6L2 Antibody (CSB-PA004261LA01HU)，的標記方式是Non-conjugated。對于ERCC6L2 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-PA004261LB01HU	ERCC6L2 Antibody, HRP conjugated	ELISA
FITC	CSB-PA004261LC01HU	ERCC6L2 Antibody, FITC conjugated
Biotin	CSB-PA004261LD01HU	ERCC6L2 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, IHC

推薦稀釋比：

Application	Recommended Dilution
IHC	1:20-1:200

Protocols：

ELISA 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.

功能：

May be involved in early DNA damage response.
基因功能參考文獻：
1. ERCC6L2-associated disorder has recently been described. We identified an additional case through whole-exome sequencing. At the age of 9 years, the patient underwent whole exome sequencing and was discovered to have a homozygous stop mutation in ERCC6L2 (NCBI RefSeq NG_034107.1), c.1687C>T (p.Arg563*). PMID: 29633571
2. The inherited bone marrow failure syndrome caused by biallelic variants in ERCC6L2 can be considered as a primary transcription deficiency rather than a DNA repair defect PMID: 29987015
3. Hebo is ubiquitously expressed, localized in the nucleus, and rapidly recruited to DNAdsb's in an NBS1-dependent manner. PMID: 27185855
4. These observations identify a distinct bone-marrow-failure syndrome due to mutations in ERCC6L2, a gene implicated in DNA repair and mitochondrial function. PMID: 24507776
相關疾病：

Bone marrow failure syndrome 2 (BMFS2)
亞細胞定位：

Nucleus. Cytoplasm, cytoskeleton, microtubule organizing center, centrosome. Mitochondrion. Note=Colocalizes with NEK6 in the centrosome. In response to DNA damage, translocates from the cytosol to mitochondria and nucleus in a reactive oxygen species (ROS)-dependent manner.
蛋白家族：

SNF2/RAD54 helicase family
組織特異性：

Expressed in bone marrow (at protein level).
數據庫鏈接：

HGNC: 26922

OMIM: 615667

KEGG: hsa:375748

STRING: 9606.ENSP00000288985

UniGene: Hs.432364

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