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  • 产品名称:COL6A2, Polyclonal Antibody

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简单介绍:
COL6A2, Polyclonal Antibody
详情介绍:
Product Name

COL6A2, Polyclonal Antibody

Full Product Name

COL6A2, CT (COL6A2, Collagen alpha-2(VI) chain)

Product Synonym Names
Anti -COL6A2, CT (COL6A2, Collagen alpha-2(VI) chain)
Product Gene Name

anti-COL6A2 antibody

[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
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Chromosome Location
Chromosome: 21; NC_000021.8 (47518033..47552763). Location: 21q22.3
OMIM
120240
3D Structure
ModBase 3D Structure for P12110
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human
Specificity
Human
Purity/Purification
Affinity Purified
Purified by Protein A affinity chromatography.
Form/Format
Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.
Immunogen
COL6A2 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 718-748 amino acids from the C-terminal region of human COL6A2.
Preparation and Storage
May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Other Notes
Small volumes of anti-COL6A2 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-COL6A2 antibody
COL6A2 is one of the three alpha chains of type VI collagen, a beaded filament collagen found in most connective tissues. The product of this gene contains several domains similar to von Willebrand Factor type A domains. These domains have been shown to bind extracellular matrix proteins, an interaction that explains the importance of this collagen in organizing matrix components. Mutations in this gene are associated with Bethlem myopathy and Ullrich scleroatonic muscular dystrophy. Three transcript variants have been identified for this gene. [provided by RefSeq].
Product Categories/Family for anti-COL6A2 antibody
Antibodies; Abs to Collagen
Applications Tested/Suitable for anti-COL6A2 antibody
ELISA (EL/EIA), Western Blot (WB)
Application Notes for anti-COL6A2 antibody
Suitable for use in Western Blot, ELISA
Dilution: ELISA: 1:1,000
Western Blot: 1:100-500
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NCBI/Uniprot data below describe general gene information for COL6A2. It may not necessarily be applicable to this product.
NCBI GI #
115527070
NCBI GeneID
1292
NCBI Accession #
NP_478055.2 [Other Products]
NCBI GenBank Nucleotide #
NM_058175.2 [Other Products]
UniProt Primary Accession #
P12110 [Other Products]
UniProt Secondary Accession #
Q13909; Q13910; Q13911; Q14048; Q14049; Q16259; Q16597; Q6P0Q1; Q9UML3; Q9Y4S8[Other Products]
UniProt Related Accession #
P12110[Other Products]
Molecular Weight
108,579 Da[Similar Products]
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NCBI Official Full Name
collagen alpha-2(VI) chain isoform 2C2a'
NCBI Official Synonym Full Names
collagen, type VI, alpha 2
NCBI Official Symbol
COL6A2  [Similar Products]
NCBI Official Synonym Symbols
PP3610
  [Similar Products]
NCBI Protein Information
collagen alpha-2(VI) chain; collagen alpha-2(VI) chain; collagen VI, alpha-2 polypeptide; human mRNA for collagen VI alpha-2 C-terminal globular domain
UniProt Protein Name
Collagen alpha-2(VI) chain
Protein Family
Collagen
UniProt Gene Name
COL6A2  [Similar Products]
UniProt Entry Name
CO6A2_HUMAN
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NCBI Summary for COL6A2
This gene encodes one of the three alpha chains of type VI collagen, a beaded filament collagen found in most connective tissues. The product of this gene contains several domains similar to von Willebrand Factor type A domains. These domains have been shown to bind extracellular matrix proteins, an interaction that explains the importance of this collagen in organizing matrix components. Mutations in this gene are associated with Bethlem myopathy and Ullrich scleroatonic muscular dystrophy. Three transcript variants have been identified for this gene. [provided by RefSeq, Jul 2008]
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UniProt Comments for COL6A2
Function: Collagen VI acts as a cell-binding protein.

Subunit structure: Trimers composed of three different chains: alpha-1(VI), alpha-2(VI), and alpha-3(VI) or alpha-5(VI) or alpha-6(VI). Interacts with CSPG4. Ref.14

Subcellular location: Secreted › extracellular space › extracellular matrix. Membrane; Peripheral membrane protein. Note: Recruited on membranes by CSPG4. Ref.13

Post-translational modification: Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.

Involvement in disease: Bethlem myopathy (BM) [MIM:158810]: A benign autosomal dominant proximal myopathy characterized by early childhood onset and joint contractures most frequently affecting the elbows and ankles.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.17 Ref.18 Ref.20 Ref.21Ullrich congenital muscular dystrophy (UCMD) [MIM:254090]: UCMD is a congenital myopathy characterized by muscle weakness and multiple joint contractures, generally noted at birth or early infancy. The clinical course is more severe than in Bethlem myopathy.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.19 Ref.20Myosclerosis autosomal recessive (MYOSAR) [MIM:255600]: A condition characterized by chronic inflammation of skeletal muscle with hyperplasia of the interstitial connective tissue. The clinical picture includes slender muscles with firm 'woody' consistency and restriction of movement of many joints because of muscle contractures.Note: The disease is caused by mutations affecting the gene represented in this entry.

Sequence similarities: Belongs to the type VI collagen family.Contains 3 VWFA domains.
Research Articles on COL6A2
1. COL6A2 is overexpressed in Down syndrome-affected umbilical cords at early and term gestational ages.
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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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