ERCC6L2 Antibody, Biotin conjugated

Datasheet

Code	CSB-PA004261LD01HU
Size	US$166
	Order now
Have Questions?	Leave a Message or Start an on-line Chat

Product Details
Related Products
Customer Reviews and Q&A
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

Biotin

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

Protocols

ELISA 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.

Customer Reviews and Q&A

■ Customer Reviews

There are currently no reviews for this product.

Submit a Review here

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

Promotions

Transmembrane Protein Questionnaire with Gifts

Email: [email protected]
Distributors Worldwide

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?

Call us

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

[email protected]

Address

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

Join us with

Subscribe newsletter

Leave a message

Message

Product >> Product Name：
Product >> Code：
Name： *
Email： *
Company/Organization：
Phone：
Since emails may get flagged by spam filters occasionally, we recommend leaving your phone number as a precaution. Your information is safe with us.
Question/Comment： *
Your Region： *
I'd like to receive email newsletters from Cusabio

Yes

No
How do you know CUSABIO?

Email

Distributor

Search engine

Advertisement

Others
CAPTCHA verification：

Place an order now

I. Product details

Product name *

Code *

Size *

Quantity *

Message

II. Contact details

Name *

Email address *

Company/Organization

Phone

III. Ship To

Address *

City *

State/Province *

Zip/Post code *

Country *

Attn Name *

Phone# *

IV. Bill To Same as Shipping Address