Rabbit anti Human collagen IV antibodyrecogizes 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 (
Nyberg et al. 2008).
Rabbit anti Human collagen IV antibody exhibits the following reactivities with Collagen IV and related proteins:
Human collagen IV | 100% |
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 (
Shah et al. 2010). Hereditary angiopathy with nephropathy aneurysms and muscle cramps (
HANAC), characterized by large renal cysts and hematuria (
Plaisier et al. 2010). Porencephaly 1 (
POREN1), a neurologic disease characterized by fluid filled cysts within the cerebral hemispheres, visual defect and facial paresis (
Gould et al. 2005). Schizencephaly (
SCHZC) presenting with full thickness cleft within the cereral hemispheres, some of the hemispheres may also be absent, replaced by cerebrospinal fluid (
Yoneda et al. 2013). Other conditions resulting from mutations in the COL4A1 gene also exist presenting with some of the features described for the above conditions.