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COL4A6, Polyclonal Antibody

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产品名称: COL4A6, Polyclonal Antibody
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简单介绍

COL4A6, Polyclonal Antibody


COL4A6, Polyclonal Antibody  的详细介绍
Product Name

COL4A6, Polyclonal Antibody

Full Product Name

COL4A6 Antibody (Center)

Product Synonym Names
Collagen alpha-6(IV) chain; COL4A6
Product Gene Name

anti-COL4A6 antibody

[Similar Products]
Antibody/Peptide Pairs
COL4A6 peptide (MBS9222453) is used for blocking the activity of COL4A6 antibody (MBS9209566)
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
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Immunogen Sequence Positions
830-859
OMIM
300914
3D Structure
ModBase 3D Structure for Q14031
Clonality
Polyclonal
Isotype
Rabbit Ig
Host
Rabbit
Species Reactivity
Human
Specificity
This COL4A6 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 830-859 amino acids from the Central region of human COL4A6.
Purity/Purification
Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)
Form/Format
Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.
Concentration
Vial Concentration: 0.5 (lot specific)
Antigen Type
Synthetic Peptide
Antigen Source
HUMAN
Preparation and Storage
Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.
Other Notes
Small volumes of anti-COL4A6 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Related Product Information for
anti-COL4A6 antibody
This gene encodes one of the six subunits of type IV
collagen, the major structural component of basement membranes.
Like the other members of the type IV collagen gene family, this
gene is organized in a head-to-head conformation with another type
IV collagen gene, alpha 5 type IV collagen, so that the gene pair
shares a common promoter. Deletions in the alpha 5 gene that extend
into the alpha 6 gene result in diffuse leiomyomatosis accompanying
the X-linked Alport syndrome caused by the deletion in the alpha 5
gene. Two splice variants have been identified for this gene.
Product Categories/Family for anti-COL4A6 antibody
Signal Transduction
Applications Tested/Suitable for anti-COL4A6 antibody
Western Blot (WB), ELISA (EIA)
Application Notes for anti-COL4A6 antibody
WB~~1:1000

Western Blot (WB) of anti-COL4A6 antibody
COL4A6 Antibody (Center) western blot analysis in A549 cell line lysates (35ug/lane).This demonstrates the COL4A6 antibody detected the COL4A6 protein (arrow).
anti-COL4A6 antibody Western Blot (WB) (WB) image
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NCBI/Uniprot data below describe general gene information for COL4A6. It may not necessarily be applicable to this product.
NCBI GI #
148536823
NCBI GeneID
1288
NCBI Accession #
NP_001838.2 [Other Products]
NCBI Related Accession #
HumanNP_378667.1[Other Products]
NCBI GenBank Nucleotide #
NM_001847.3 [Other Products]
UniProt Primary Accession #
Q14031 [Other Products]
UniProt Secondary Accession #
Q12823; Q14053; Q5JYH6; Q5JYH8; Q9NQM5; Q9NTX3; Q9UJ76; Q9UMG6; Q9Y4L4[Other Products]
UniProt Related Accession #
Q14031[Other Products]
Molecular Weight
163807
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NCBI Official Full Name
collagen alpha-6(IV) chain isoform A
NCBI Official Synonym Full Names
collagen, type IV, alpha 6
NCBI Official Symbol
COL4A6  [Similar Products]
NCBI Official Synonym Symbols
DFNX6; DELXq22.3; CXDELq22.3
  [Similar Products]
NCBI Protein Information
collagen alpha-6(IV) chain
UniProt Protein Name
Collagen alpha-6(IV) chain
Protein Family
Collagen
UniProt Gene Name
COL4A6  [Similar Products]
UniProt Entry Name
CO4A6_HUMAN
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NCBI Summary for COL4A6
This gene encodes one of the six subunits of type IV collagen, the major structural component of basement membranes. Like the other members of the type IV collagen gene family, this gene is organized in a head-to-head conformation with another type IV collagen gene, alpha 5 type IV collagen, so that the gene pair shares a common promoter. Deletions in the alpha 5 gene that extend into the alpha 6 gene result in diffuse leiomyomatosis accompanying the X-linked Alport syndrome caused by the deletion in the alpha 5 gene. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Dec 2013]
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UniProt Comments for COL4A6
COL4A6: Type IV collagen is the major structural component of glomerular basement membranes (GBM), forming a 'chicken-wire' meshwork together with laminins, proteoglycans and entactin/nidogen. Deletions covering the N-terminal regions of COL4A5 and COL4A6, which are localized in a head-to-head manner, are found in the chromosome Xq22.3 centromeric deletion syndrome. This results in a phenotype with features of diffuse leiomyomatosis and Alport syndrome (DL-ATS). Belongs to the type IV collagen family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Extracellular matrix; Secreted, signal peptide; Secreted

Chromosomal Location of Human Ortholog: Xq22

Cellular Component: endoplasmic reticulum lumen; collagen type IV; extracellular region

Molecular Function: extracellular matrix structural constituent

Biological Process: extracellular matrix disassembly; collagen catabolic process; extracellular matrix organization and biogenesis; cell adhesion

Disease: Deafness, X-linked 6
Product References and Citations for anti-COL4A6 antibody
Romero, R., et al. Am. J. Obstet. Gynecol. (2010) In press :
Bailey, S.D., et al. Diabetes Care (2010) In press :
Romero, R., et al. Am. J. Obstet. Gynecol. 202 (5), 431 (2010) :
Talmud, P.J., et al. Am. J. Hum. Genet. 85(5):628-642(2009)
Skovbjerg, H., et al. BMC Cancer 9, 136 (2009) :

Research Articles on COL4A6
1. COL4A6 is associated with X-linked nonsyndromic hearing loss.
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Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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