ERCC6L2 Antibody

Datasheet

Code	CSB-PA004261LA01HU
Size	US$166
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Image	Immunohistochemistry of paraffin-embedded human lung tissue using CSB-PA004261LA01HU at dilution of 1:100
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Product Details
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Target Background

Product Details

Full Product Name

Rabbit anti-Homo sapiens (Human) ERCC6L2 Polyclonal antibody

Uniprot No.

Q5T890

Target Names

ERCC6L2

Alternative Names

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

Raised in

Rabbit

Species Reactivity

Human

Immunogen

Recombinant Human DNA excision repair protein ERCC-6-like 2 protein (72-371AA)

Immunogen Species

Homo sapiens (Human)

Conjugate

Non-conjugated

The ERCC6L2 Antibody (Product code: CSB-PA004261LA01HU) is Non-conjugated. For ERCC6L2 Antibody with conjugates, please check the following table.

Conjugate	Product Code	Product Name	Application
HRP	CSB-PA004261LB01HU	ERCC6L2 Antibody, HRP conjugated	ELISA
FITC	CSB-PA004261LC01HU	ERCC6L2 Antibody, FITC conjugated
Biotin	CSB-PA004261LD01HU	ERCC6L2 Antibody, Biotin conjugated	ELISA

Clonality

Polyclonal

Isotype

IgG

Purification Method

>95%, Protein G purified

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4

Form

Liquid

Tested Applications

ELISA, IHC

Recommended Dilution

Application	Recommended Dilution
IHC	1:20-1:200

Protocols

ELISA Protocol
Immunohistochemistry (IHC) Protocol

Troubleshooting and FAQs

Antibody FAQs

Storage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Lead Time

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.

Usage

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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Target Background

Function

May be involved in early DNA damage response.

Gene References into Functions

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
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
Hebo is ubiquitously expressed, localized in the nucleus, and rapidly recruited to DNAdsb's in an NBS1-dependent manner. PMID: 27185855
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

Involvement in disease

Bone marrow failure syndrome 2 (BMFS2)

Subcellular Location

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.

Protein Families

SNF2/RAD54 helicase family

Tissue Specificity

Expressed in bone marrow (at protein level).

Database Links

HGNC: 26922

OMIM: 615667

KEGG: hsa:375748

STRING: 9606.ENSP00000288985

UniGene: Hs.432364

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Service

Phage Display Service

Antibody Service

Protein Service

Gene Synthesis Service

Oligo Synthesis Service

Antibody FAQs What Is an Antibody? How to Choose an Antibody for Scientific Research? The difference between primary antibody and secondary antibod The Meaning of Irregular Antibody Screening Tag Antibodies, Your Good Partner in Protein Research How to Choose the Loading Control Antibodies?

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301-363-4651 (Available 9 a.m. to 5 p.m. CST from Monday to Friday)

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Address

7505 Fannin St., Ste 610, Room 7 (CUBIO Innovation Center), Houston, TX 77054, USA

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