protein

Recombinant Human Collagen Type IV Alpha 3

MBS142942

0.002 mg

140 USD

Recombinant Protein

Recombinant Human Collagen Type IV Alpha 3

Collagen Type IV Alpha 3

COL4A3 Human; Collagen Type IV Alpha 3 Human Recombinant; Collagen alpha-3(IV) chain; Goodpasture antigen; COL4A3; Glomerular Basal Membrane; GBM

collagen alpha-3(IV) chain; Collagen alpha-3(IV) chain; collagen alpha-3(IV) chain; collagen alpha-3(IV) chain; collagen IV, alpha-3 polypeptide; tumstatin; collagen, type IV, alpha 3 (Goodpasture antigen); Goodpasture antigenTumstatin

COL4A3

COL4A3; COL4A3

CO4A3_HUMAN

Sf9 Insect Cells

1670

The protein solution (0.6 mg/ml) contains 20mM Hepes, pH 8.0, and 4M Urea. Sterile Filtered clear solution.

0.12-0.5 ug/ml (depending on the type of ELISA plate and coating buffer). Suitable for biotinylation and iodination.

Recommendations for storage buffer: Ionic strength between 50 and 100 mM, neutral to slightly alkaline pH and 4 M urea as dissociating agent. Storage temperature is -70 degree to -80 degree C.Please prevent freeze-thaw cycles.

Caution: It has been reported that the immunodominant epitope of COL4A3 is a cryptic epitope that is not easily accessible to the corresponding autoantibodies. It is necessary to treat the protein under nonreducing conditions with a denaturant such as urea to unmask the epitopes (see Hellmark et al. in Autoantibodies, Peter, J.B. and Shoenfeld, Y., eds., Elsevier B.V., 1996, pp 291-298).

RECOMBINANT & NATURAL PROTEINS; Recombinant Proteins; Collagen

Description: Human a3 chain of collagen IV; identical with the antigen called "glomerular basal membrane antigen" (GBM). Recombinant antigen for solid (ELISA) and fluid phase diagnostic assays. Binds IgG-type human auto-antibodies. Calculated Molecular weight: 43,591 Dalton. Calculated isoelectric point: pH 8.9. cDNA coding for a minicollagen version of the human collagen IV a3 chain fused to a hexa-histidine purification tag. The term minicollagen designates the removal of most of the epitope-less triplehelical collagenous region (situated between the N-terminal 7S domain and the C-terminal noncollagenous NC1 domain), which is a requirement for recombinant production of this antigen. Introduction: Type IV collagen is a major structural component of basement membranes. It is a multimeric protein composed of 3 alpha subunits, which are encoded by 6 different genes, alpha 1 through alpha 6. Each of these alpha subunits can form a triple helix structure with 2 other subunits to form type IV collagen. The Goodpasture syndrome is a condition in which autoantibodies bind to the collagen molecules in the basement membranes of alveoli and glomeruli. The epitopes that elicit these autoantibodies are restricted basically to the non-collagenous C-terminal domain of the protein. There are numerous alternative transcripts that appear to be unique to the human COL4A3 and alternative splicing is limited to the six exons that encode this C-terminal domain. COL4A3 is also linked to an autosomal recessive form of Alport syndrome. The mutations contributing to the Alport syndrome are also situated within the exons that encode this C-terminal region. COL4A3 is organized in a head-to-head conformation with another type IV collagen gene so that each gene pair shares a common promoter. Several exons of COL4A3 are interspersed with exons of an uncharacterized gene which is on the opposite strand.

89142730

NP_000082.2

NM_000091.4

Q01955

135,079 Da

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)

Type IV collagen, the major structural component of basement membranes, is a multimeric protein composed of 3 alpha subunits. These subunits are encoded by 6 different genes, alpha 1 through alpha 6, each of which can form a triple helix structure with 2 other subunits to form type IV collagen. This gene encodes alpha 3. In the Goodpasture syndrome, autoantibodies bind to the collagen molecules in the basement membranes of alveoli and glomeruli. The epitopes that elicit these autoantibodies are localized largely to the non-collagenous C-terminal domain of the protein. A specific kinase phosphorylates amino acids in this same C-terminal region and the expression of this kinase is upregulated during pathogenesis. This gene is also linked to an autosomal recessive form of Alport syndrome. The mutations contributing to this syndrome are also located within the exons that encode this C-terminal region. 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. [provided by RefSeq, Jun 2010]

COL4A3: 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. Autoantibodies against the NC1 domain of alpha 3(IV) are found in Goodpasture syndrome, an autoimmune disease of lung and kidney. Defects in COL4A3 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 COL4A3 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. Defects in COL4A3 are a cause of Alport syndrome autosomal dominant (APSAD). Alport syndrome 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. Belongs to the type IV collagen family. 5 isoforms of the human protein are produced by alternative splicing. Protein type: Secreted, signal peptide; Secreted. Chromosomal Location of Human Ortholog: 2q36-q37. Cellular Component: endoplasmic reticulum lumen; collagen type IV; extracellular region; basement membrane. Molecular Function: metalloendopeptidase inhibitor activity; integrin binding; protein binding; extracellular matrix structural constituent; structural molecule activity. Biological Process: caspase activation; axon guidance; extracellular matrix organization and biogenesis; blood circulation; glomerular basement membrane development; cell proliferation; extracellular matrix disassembly; collagen catabolic process; negative regulation of cell proliferation; negative regulation of angiogenesis; sensory perception of sound; cell surface receptor linked signal transduction; cell adhesion. Disease: Hematuria, Benign Familial; Alport Syndrome, Autosomal Dominant; Alport Syndrome, Autosomal Recessive

0.002 mg

140 USD

0.01 mg

205

1 mg

5015