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Collagen IV alpha4, Antibody

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產(chǎn)品名稱: Collagen IV alpha4, Antibody
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簡單介紹

Collagen IV alpha4, Antibody


Collagen IV alpha4, Antibody  的詳細(xì)介紹
Product Name

Collagen IV alpha4 (COL4A4), Antibody

Full Product Name

Rabbit Collagen IV alpha4 Antibody

Product Synonym Names
Collagen IV a4
Product Gene Name

anti-COL4A4 antibody

[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
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Chromosome Location
Chromosome: 2; NC_000002.11 (227867427..228029275, complement). Location: 2q35-q37
OMIM
120131
3D Structure
ModBase 3D Structure for P53420
Host
Rabbit
Species Reactivity
Human
Specificity
Collagen IV alpha4 antibody detects endogenous levels of total Collagen IV alpha4 protein.
Form/Format
Phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol
Concentration
1 mg/ml (lot specific)
Other Notes
Small volumes of anti-COL4A4 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-COL4A4 antibody
Immunofluorescence (IF), Immunohistochemistry (IHC), ELISA (EIA)
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NCBI/Uniprot data below describe general gene information for COL4A4. It may not necessarily be applicable to this product.
NCBI GI #
116256356
NCBI GeneID
1286
NCBI Accession #
NP_000083.3 [Other Products]
NCBI GenBank Nucleotide #
NM_000092.4 [Other Products]
UniProt Primary Accession #
P53420 [Other Products]
UniProt Secondary Accession #
Q53RW9; Q53S42; Q53WR1; A8MTZ1[Other Products]
UniProt Related Accession #
P53420[Other Products]
Molecular Weight
164,038 Da[Similar Products]
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NCBI Official Full Name
collagen alpha-4(IV) chain
NCBI Official Synonym Full Names
collagen, type IV, alpha 4
NCBI Official Symbol
COL4A4??[Similar Products]
NCBI Official Synonym Symbols
CA44
??[Similar Products]
NCBI Protein Information
collagen alpha-4(IV) chain; collagen alpha-4(IV) chain; Collagen IV, alpha-4 polypeptide; collagen of basement membrane, alpha-4 chain
UniProt Protein Name
Collagen alpha-4(IV) chain
Protein Family
Collagen
UniProt Gene Name
COL4A4??[Similar Products]
UniProt Entry Name
CO4A4_HUMAN
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NCBI Summary for COL4A4
This gene encodes one of the six subunits of type IV collagen, the major structural component of basement membranes. This particular collagen IV subunit, however, is only found in a subset 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 so that each gene pair shares a common promoter. Mutations in this gene are associated with type II autosomal recessive Alport syndrome (hereditary glomerulonephropathy) and with familial benign hematuria (thin basement membrane disease). Two transcripts, differing only in their transcription start sites, have been identified for this gene and, as is common for collagen genes, multiple polyadenylation sites are found in the 3' UTR. [provided by RefSeq, Jul 2008]
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UniProt Comments for COL4A4
COL4A4: 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. Defects in COL4A4 are a cause of Alport syndrome autosomal recessive (APSAR). APSAR is characterized by progressive glomerulonephritis, glomerular basement membrane defects, renal failure, sensorineural deafness and specific eye abnormalities (lenticonous and macular flecks). The disorder shows considerable heterogeneity in that families differ in the age of end-stage renal disease and the occurrence of deafness. Defects in COL4A4 are a cause of benign familial hematuria (BFH); also known as thin basement membrane nephropathy. BFH is characterized by persistent hematuria, an electron microscopically detectable thin glomerular basement membrane (GBM) and an autosomal dominant mode of inheritance. Renal function remains normal. In children, differentiation between BFH and AS can be difficult, because both disorders are manifested by persistent hematuria and thin GBM at that age. Belongs to the type IV collagen family.

Protein type: Secreted, signal peptide; Secreted

Chromosomal Location of Human Ortholog: 2q35-q37

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

Molecular Function: extracellular matrix structural constituent

Biological Process: axon guidance; extracellular matrix disassembly; collagen catabolic process; extracellular matrix organization and biogenesis; glomerular basement membrane development

Disease: Alport Syndrome, Autosomal Recessive
Research Articles on COL4A4
1. Our findings suggest that variants in the COL4A4 gene may contribute to the development of lattice degeneration of the retina.
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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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