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Argininemia is an autosomal recessive urea cycle disorder where a deficiency of the enzyme arginase causes a buildup of arginine and ammonia in the blood. Ammonia, which is formed when proteins are broken down in the body, is toxic if levels become too high; the nervous system is especially sensitive to the effects of excess ammonia.
11846 Ensembl ENSG00000118520 ENSMUSG00000019987 UniProt P05089 Q61176 RefSeq (mRNA) NM_000045 NM_001244438 NM_001369020 NM_007482 RefSeq (protein) NP_000036 NP_001231367 NP_001355949 NP_031508 Location (UCSC) Chr 6: 131.47 – 131.58 Mb Chr 10: 24.79 – 24.8 Mb PubMed search Wikidata View/Edit Human View/Edit Mouse The human ARG1 gene encodes the protein arginase. Function Arginase catalyzes ...
Only the l-arginine (symbol Arg or R) enantiomer is found naturally. [1] Arg residues are common components of proteins. It is encoded by the codons CGU, CGC, CGA, CGG, AGA, and AGG. [2] The guanidine group in arginine is the precursor for the biosynthesis of nitric oxide. [3] Like all amino acids, it is a white, water-soluble solid.
Synthesis of NAcGlu by N-acetylglutamate synthase (NAGS) is stimulated by both Arg, allosteric stimulator of NAGS, and Glu, a product in the transamination reactions and one of NAGS's substrates, both of which are elevated when free amino acids are elevated. So Glu not only is a substrate for NAGS but also serves as an activator for the urea cycle.
In enzymology, an arginine—tRNA ligase (EC 6.1.1.19) is an enzyme that catalyzes the chemical reaction. ATP + L-arginine + tRNAArg AMP + diphosphate + L-arginyl-tRNAArg. The 3 substrates of this enzyme are ATP, L-arginine, and tRNA(Arg), whereas its 3 products are AMP, diphosphate, and L-arginyl-tRNA(Arg).
384 11847 Ensembl ENSG00000081181 ENSMUSG00000021125 UniProt P78540 O08691 RefSeq (mRNA) NM_001172 NM_009705 RefSeq (protein) NP_001163 NP_033835 Location (UCSC) Chr 14: 67.62 – 67.65 Mb Chr 12: 79.18 – 79.2 Mb PubMed search Wikidata View/Edit Human View/Edit Mouse Arginase, type II is an arginase protein that in humans is encoded by the ARG2 gene. Function Arginase catalyzes the ...
Arginylation is a post-translational modification in which proteins are modified by the addition of arginine (Arg) at the N-terminal amino group or side chains of reactive amino acids by the enzyme, arginyltransferase (ATE1). Recent studies have also revealed that hundreds of proteins in vivo are arginylated, proteins which are essential for ...
In molecular biology, the arginine repressor (ArgR) is a repressor of prokaryotic arginine deiminase pathways.. The arginine dihydrolase (AD) pathway is found in many prokaryotes and some eukaryotes, an example of the latter being Giardia lamblia (Giardia intestinalis). [1]