SERPIN A8 (AGT) Human Cellexp Recombinant Protein

The protein encoded by this gene, pre-angiotensinogen or angiotensinogen precursor, is expressed in the liver and is cleaved by the enzyme renin in response to lowered blood pressure. The resulting product, angiotensin I, is then cleaved by angiotensin converting enzyme (ACE) to generate the physiologically active enzyme angiotensin II. The protein is involved in maintaining blood pressure and in the pathogenesis of essential hypertension and preeclampsia. Mutations in this gene are associated with susceptibility to essential hypertension, and can cause renal tubular dysgenesis, a severe disorder of renal tubular development. Defects in this gene have also been associated with non-familial structural atrial fibrillation, and inflammatory bowel disease. [provided by RefSeq, Jul 2008]

angiotensin: Essential component of the renin-angiotensin system (RAS), a potent regulator of blood pressure, body fluid and electrolyte homeostasis. In response to lowered blood pressure, the enzyme renin cleaves angiotensinogen to produce angiotensin-1 (angiotensin 1-10). Angiotensin-1 is a substrate of ACE (angiotensin converting enzyme) that removes a dipeptide to yield the physiologically active peptide angiotensin-2 (angiotensin 1- 8). Angiotensin-1 and angiotensin-2 can be further processed to generate angiotensin-3 (angiotensin 2-8), angiotensin-4 (angiotensin 3-8). Angiotensin 1-7 is cleaved from angiotensin-2 by ACE2 or from angiotensin-1 by MME (neprilysin). Angiotensin 1-9 is cleaved from angiotensin-1 by ACE2. Genetic variations in AGT are a cause of susceptibility to essential hypertension (EHT). Essential hypertension is a condition in which blood pressure is consistently higher than normal with no identifiable cause. Defects in AGT are a cause of renal tubular dysgenesis (RTD). RTD is an autosomal recessive severe disorder of renal tubular development characterized by persistent fetal anuria and perinatal death, probably due to pulmonary hypoplasia from early-onset oligohydramnios (the Potter phenotype). Belongs to the serpin family.

Protein type: Secreted; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 1q42.2

Cellular Component: extracellular space; cytoplasm; extracellular region

Molecular Function: serine-type endopeptidase inhibitor activity; protein binding; sodium channel regulator activity; growth factor activity; hormone activity; type 2 angiotensin receptor binding; superoxide-generating NADPH oxidase activator activity; type 1 angiotensin receptor binding

Biological Process: extracellular matrix organization and biogenesis; renal system process; positive regulation of nitric oxide biosynthetic process; establishment of blood-nerve barrier; negative regulation of nerve growth factor receptor signaling pathway; positive regulation of transcription, DNA-dependent; stress-activated MAPK cascade; female pregnancy; positive regulation of multicellular organism growth; positive regulation of vasodilation; ovarian follicle rupture; activation of NF-kappaB transcription factor; positive regulation of fibroblast proliferation; cell-cell signaling; positive regulation of superoxide release; negative regulation of neuron apoptosis; kidney development; positive regulation of NAD(P)H oxidase activity; positive regulation of cytokine production; angiotensin mediated regulation of renal output; response to muscle activity involved in regulation of muscle adaptation; regulation of calcium ion transport; regulation of norepinephrine secretion; negative regulation of tissue remodeling; positive regulation of phosphoinositide 3-kinase cascade; positive regulation of peptidyl-tyrosine phosphorylation; angiotensin mediated vasoconstriction involved in regulation of systemic arterial blood pressure; phospholipase C activation; regulation of transmission of nerve impulse; regulation of vasoconstriction; smooth muscle cell differentiation; G-protein signaling, coupled to IP3 second messenger (phospholipase C activating); cytokine secretion; nitric oxide mediated signal transduction; regulation of long-term neuronal synaptic plasticity; peristalsis; cell-matrix adhesion; renin-angiotensin regulation of aldosterone production; positive regulation of cellular protein metabolic process; smooth muscle cell proliferation; cellular lipid metabolic process; angiotensin maturation; excretion; vasodilation; response to salt stress; negative regulation of cell proliferation; fibroblast proliferation; positive regulation of MAPKKK cascade; renin-angiotensin regulation of blood vessel size; regulation of blood pressure; positive regulation of epidermal growth factor receptor signaling pathway; renin-angiotensin regulation of blood volume; regulation of cell growth; angiotensin mediated drinking behavior; artery smooth muscle contraction; aging; positive regulation of fatty acid biosynthetic process; blood vessel development; cellular sodium ion homeostasis; renal response to blood flow during renin-angiotensin regulation of systemic arterial blood pressure; activation of NF-kappaB-inducing kinase; positive regulation of peptidyl-serine phosphorylation; positive regulation of organ growth; regulation of cell proliferation; G-protein coupled receptor protein signaling pathway; negative regulation of angiogenesis; cellular protein metabolic process; ureteric bud branching; blood vessel remodeling; G-protein signaling, coupled to cGMP nucleotide second messenger; response to cold; negative regulation of cell growth; astrocyte activation; positive regulation of inflammatory response

Disease: Renal Tubular Dysgenesis; Hypertension, Essential