Spécificité
This antibody recognises human collagen IV, also known as Collagen alpha-1(IV) chain. Collagen IV is a 1497 amino acid structural extracellular matrix protein and key component of the glomerular basement membrane. Arresten, comprising the C-terminal 225 amino acids of collagen IV demonstrates potent anti tumorigenic and anti angiogenic proterties. Rabbit anti Human collagen IV antibody exhibits the following reactivities with Collagen IV and related proteins:Human collagen IV100% Human types I, III and V< 10% Human collagen II<1% Human plasma proteins do not interfere with collagen binding.Defects in the COL4A1 gene encoding collagen 4 can lead to the presentation of a variety of conditions including Brain small vessel disease with or without ocular anomalies (BSVD), demonstrating weakened brain blood vessels and retinal arteriolar tortuosity. Hereditary angiopathy with nephropathy aneurysms and muscle cramps (HANAC), characterized by large renal cysts and hematuria. Porencephaly 1 (POREN1), a neurologic disease characterized by fluid filled cysts within the cerebral hemispheres, visual defect and facial paresis. Schizencephaly (SCHZC) presenting with full thickness cleft within the cereral hemispheres, some of the hemispheres may also be absent, replaced by cerebrospinal fluid. Other conditions resulting from mutations in the COL4A1 gene also exist presenting with some of the features described for the above conditions.