background:
Arginase I which is expressed almost exclusively in the liver, catalyzes the conversion of arginine to ornithine and urea . The human arginase I gene, which maps to chromosome 6q23, encodes a 322 amino acid protein. Arginase I exists as a homotrimeric protein and contains a binuclear manganese cluster. Arginase II catalyzes the same reaction as arginase I, but differs in its tissue specificity and subcellular location. Specifically, arginase II localizes to the mitochondria. Arginase II is expressed in non-hepatic tissues, with the highest levels of expression in the kidneys, but, unlike arginase I, is not expressed in liver. The human arginase II gene, which maps to chromosome 14q24.1-q24.3, encodes a 354 amino acid protein. In addition, arginase II contains a putative amino-terminal mitochondrial localization sequence.
Subunit:
Homotrimer.
Subcellular Location:
Cytoplasm.
DISEASE:
Defects in ARG1 are the cause of argininemia (ARGIN) ; also known as hyperargininemia. Argininemia is 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, progressive spastic quadriplegia.
Similarity:
Belongs to the arginase family.
Database links:
Entrez Gene: 51728 Cow
Entrez Gene: 383 Human
Entrez Gene: 11846 Mouse
Entrez Gene: 397115 Pig
Entrez Gene: 100008814 Rabbit
Entrez Gene: 29221 Rat
Omim: 608313 Human
SwissProt: Q2KJ64 Cow
SwissProt: P05089 Human
SwissProt: Q61176 Mouse
SwissProt: Q95JC8 Pig
SwissProt: Q95KM0 Rabbit
SwissProt: P07824 Rat
Unigene: 88934 Human
Unigene: 154144 Mouse
Unigene: 9857 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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