COL4A4-Specific Antibody | MyBioSource MBS2529785 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

COL4A4-Specific Antibody

catalog :

MBS2529785

quantity :

0.06 mL

price :

195 USD

clonality :

polyclonal

host :

rabbit

conjugate :

nonconjugated

reactivity :

human

application :

ELISA, immunocytochemistry

more info or order :

MyBioSource product webpage

image

image 1 :

Immunohistochemistry of paraffinembedded human colon cancer tissue slide with COL4A4-Specific Polyclonal Antibody at dilution of 1:200.

image 2 :

Immunofluorescent analysis of fixed HeLa cells using COL4A4-Specific Polyclonal Antibody at dilution of 1:50 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).

product information

catalog number :

MBS2529785

products type :

Antibody

products full name :

COL4A4-Specific Antibody

products short name :

[COL4A4-Specific]

products name syn :

[CA44, COL4A4, Collagen alpha 4(IV) chain,Collagen Type IV, collagen, type IV, alpha 4]

other names :

[Collagen alpha-4(IV) chain; Collagen alpha-4(IV) chain; collagen alpha-4(IV) chain; collagen alpha-4(IV) chain; Collagen IV, alpha-4 polypeptide; collagen of basement membrane, alpha-4 chain; collagen, type IV, alpha 4]

products gene name :

[COL4A4]

other gene names :

[COL4A4; COL4A4; CA44]

uniprot entry name :

CO4A4_HUMAN

clonality :

Polyclonal

isotype :

IgG

host :

Rabbit

reactivity :

Human

sequence length :

1690

purity :

Antigen affinity purification

storage stability :

Store at -20°C. Avoid freeze / thaw cycles

tested application :

IF, ELISA

app notes :

IF: 1:50-1-500

image1 heading :

Immunohistochemistry

image2 heading :

Immunofluorescent

other info1 :

Immunogen: Peptide. Calculated MW: 164kd. Buffer: PBS with 0.1% sodium azide and 50% glycerol pH 7.3.

products description :

COL4A4 belongs to the type IV collagen family. COL4A4 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). Defects in COL4A4 are a cause of benign familial hematuria (BFH). This antibody is specific to COL4A4.

ncbi gi num :

259016360

ncbi acc num :

NM_000092

ncbi pathways :

Amoebiasis Pathway (167324); Amoebiasis Pathway (167191); Assembly Of Collagen Fibrils And Other Multimeric Structures Pathway (730306); Axon Guidance Pathway (105688); Collagen Biosynthesis And Modifying Enzymes Pathway (645289); Collagen Formation Pathway (645288); Developmental Biology Pathway (477129); ECM-receptor Interaction Pathway (83068); ECM-receptor Interaction Pathway (479); Extracellular Matrix Organization Pathway (576262)

ncbi summary :

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]

uniprot summary :

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; Secreted, signal peptide. 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: extracellular matrix disassembly; axon guidance; collagen catabolic process; extracellular matrix organization and biogenesis; glomerular basement membrane development. Disease: Alport Syndrome, Autosomal Recessive

size1 :

0.06 mL

price1 :

195 USD

size2 :

0.12 mL

price2 :

270

size3 :

0.2 mL

price3 :

435

more info or order :

MyBioSource product webpage