ARG1

Arginase catalyzes the hydrolysis of arginine to ornithine and urea. At least two isoforms of mammalian arginase exist (types I and II) which differ in their tissue distribution, subcellular localization, immunologic crossreactivity and physiologic function. The type I isoform encoded by this gene, is a cytosolic enzyme and expressed predominantly in the liver as a component of the urea cycle. Inherited deficiency of this enzyme results in argininemia, an autosomal recessive disorder characterized by hyperammonemia. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Sep 2011]

Arginase 1

Key element of the urea cycle converting L-arginine to urea and L-ornithine, which is further metabolized into metabolites proline and polyamides that drive collagen synthesis and bioenergetic pathways critical for cell proliferation, respectively; the urea cycle takes place primarily in the liver and, to a lesser extent, in the kidneys.
Functions in L-arginine homeostasis in nonhepatic tissues characterized by the competition between nitric oxide synthase (NOS) and arginase for the available intracellular substrate arginine. Arginine metabolism is a critical regulator of innate and adaptive immune responses. Involved in an antimicrobial effector pathway in polymorphonuclear granulocytes (PMN). Upon PMN cell death is liberated from the phagolysosome and depletes arginine in the microenvironment leading to suppressed T cell and natural killer (NK) cell proliferation and cytokine secretion (PubMed:15546957, PubMed:16709924, PubMed:19380772).
In group 2 innate lymphoid cells (ILC2s) promotes acute type 2 inflammation in the lung and is involved in optimal ILC2 proliferation but not survival (By similarity).
In humans, the immunological role in the monocytic/macrophage/dendritic cell (DC) lineage is unsure.

Adaptive immune response Source: UniProtKB-KW
Aging Source: Ensembl
Arginine catabolic process Source: ProtInc
Arginine catabolic process to ornithine Source: GO_Central
Cellular response to dexamethasone stimulus Source: Ensembl
cellular response to glucagon stimulus Source: Ensembl
Cellular response to hydrogen peroxide Source: Ensembl
Cellular response to interleukin-4 Source: Ensembl
Cellular response to lipopolysaccharide Source: Ensembl
Cellular response to transforming growth factor beta stimulus Source: Ensembl
Collagen biosynthetic process Source: Ensembl
Defense response to protozoan Source: Ensembl
Innate immune response Source: UniProtKB-KW
Liver development Source: Ensembl
Lung development Source: Ensembl
Mammary gland involution Source: Ensembl
Maternal process involved in female pregnancy Source: Ensembl
Negative regulation of activated T cell proliferation Source: Ensembl
Negative regulation of interferon-gamma-mediated signaling pathway Source: UniProtKB
Negative regulation of T cell proliferation Source: UniProtKB
Negative regulation of T-helper 2 cell cytokine production Source: Ensembl
Neutrophil degranulation Source: Reactome
Positive regulation of endothelial cell proliferation Source: Ensembl
Positive regulation of neutrophil mediated killing of fungus Source: UniProtKB
Regulation of L-arginine import Source: Ensembl
Response to amine Source: Ensembl
Response to amino acid Source: Ensembl
Response to axon injury Source: Ensembl
Response to cadmium ion Source: Ensembl
Response to drug Source: Ensembl
Response to herbicide Source: Ensembl
Response to manganese ion Source: Ensembl
Response to methylmercury Source: Ensembl
Response to selenium ion Source: Ensembl
Response to vitamin A Source: Ensembl
Response to vitamin E Source: Ensembl
Response to zinc ion Source: Ensembl
Urea cycle Source: Reactome

Cytoplasm; Cytoplasmic granule. Localized in azurophil granules of neutrophils (PubMed:15546957).

Argininemia (ARGIN): A rare autosomal recessive disorder of the urea cycle. Arginine is elevated in the blood and cerebrospinal fluid, and periodic hyperammonemia occurs. Clinical manifestations include developmental delay, seizures, mental retardation, hypotonia, ataxia and progressive spastic quadriplegia.