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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 Antibody

Product Synonym Names
DKFZp586E1322; FLJ46862; PP3610
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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OMIM
120240
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human, Mouse, Rat
Purity/Purification
Antigen affinity purification
Immunogen
Fusion protein of COL6A2
Calculated Molecular Weight: 109kd
Observed Molecular Weight: 140kd
Buffer
PBS with 0.02% sodium azide and 50% glycerol pH 7.3.
Santa Cruz Alternative
Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-292186
Preparation and Storage
Store at -20 degree C. Avoid freeze / thaw cycles
ISO Certification
Manufactured in an ISO 9001:2015 Certified Laboratory.
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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Applications Tested/Suitable for anti-COL6A2 antibody
ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
Application Notes for anti-COL6A2 antibody
WB: 1:200-1:2000
IHC: 1:20-1:200

Testing Data of anti-COL6A2 antibody
anti-COL6A2 antibody Testing Data image
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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 Secondary Accession #
Q13909; Q13910; Q13911; Q14048; Q14049; Q16259; Q16597; Q6P0Q1; Q9UML3; Q9Y4S8[Other Products]
UniProt Related Accession #
P12110[Other Products]
Molecular Weight
87,280 Da
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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
COL6A2: Collagen VI acts as a cell-binding protein. Defects in COL6A2 are a cause of Bethlem myopathy (BM). BM is a rare autosomal dominant proximal myopathy characterized by early childhood onset (complete penetrance by the age of 5) and joint contractures most frequently affecting the elbows and ankles. Defects in COL6A2 are a cause of Ullrich congenital muscular dystrophy (UCMD); also known as Ullrich scleroatonic muscular dystrophy. UCMD is an autosomal recessive 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. Defects in COL6A2 are the cause of myosclerosis autosomal recessive (MYOSAR); also known as myosclerotic myopathy or congenital myosclerosis of Lowenthal. 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. Belongs to the type VI collagen family. 3 isoforms of the human protein are produced by alternative splicing.

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

Chromosomal Location of Human Ortholog: 21q22.3

Cellular Component: extracellular matrix; proteinaceous extracellular matrix; extracellular space; protein complex; collagen; endoplasmic reticulum lumen; extracellular region; vesicle; sarcolemma

Molecular Function: protein binding

Biological Process: collagen catabolic process; axon guidance; extracellular matrix disassembly; extracellular matrix organization and biogenesis; response to glucose stimulus; cell adhesion

Disease: Bethlem Myopathy; Ullrich Congenital Muscular Dystrophy; Myosclerosis, Autosomal Recessive
Research Articles on COL6A2
1. In UCMD, 8 mutations were identified in COL6A2 in Chinese patients.
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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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