抗体

COL4A4-Specific Antibody

MBS2529785

0.06毫升

195美元

多克隆

兔

未共轭

酶联免疫吸附测定, 免疫细胞化学

抗体

COL4A4-Specific Antibody

[COL4A4-Specific]

[CA44, COL4A4, 胶原甲4(IV)链, IV型胶原, 胶原, 类型IV, 甲4]

[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]

[COL4A4]

[COL4A4;COL4A4;CA44]

CO4A4_HUMAN

多克隆

IgG

兔

人类

1690

抗原亲和纯化

存放在-20°C。 避免反复冷冻/解冻

IF, 酶联免疫吸附测定

IF: 1:50-1-500

免疫组化

Immunofluorescent

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

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.

259016360

NM_000092

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)

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]

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

0.06毫升

195美元

0.12毫升

270

0.2毫升

435