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  • serine
  • 4. These enzyme activities increase markedly when R. spheroides is grown in the presence of glycine, glyoxylate, glycollate, oxalate or serine. (biochemj.org)
  • When coupled to serine hydroxymethyltransferase, the glycine cleavage system overall reaction becomes: 2 glycine + NAD+ + H2O → serine + CO2 + NH3 + NADH + H+ In humans and most vertebrates, the glycine cleavage system is part of the most prominent glycine and serine catabolism pathway. (wikipedia.org)
  • The glycine cleavage system takes glycine, which is created from an unwanted byproduct of the Calvin cycle, and converts it to serine which can reenter the cycle. (wikipedia.org)
  • Initial research using carbon labeling showed decreased levels of CO2 and serine production in the liver, pointing directly to deficiencies glycine cleavage reaction. (wikipedia.org)
  • Plants
  • In plants, animals and bacteria the glycine cleavage system catalyzes the following reversible reaction: Glycine + H4folate + NAD+ ↔ 5,10-methylene-H4folate + CO2 + NH3 + NADH + H+ In the enzymatic reaction, H-protein activates the P-protein, which catalyzes the decarboxylation of glycine and attaches the intermediate molecule to the H-protein to be shuttled to the T-protein. (wikipedia.org)
  • This reaction, and by extension the glycine cleavage system, is required for photorespiration in C3 plants. (wikipedia.org)
  • The glycine cleavage system is constantly present in the leaves of plants, but in small amounts until they are exposed to light. (wikipedia.org)
  • present
  • 6. The increase in the activity of glycine decarboxylase on illumination of R. spheroides in a medium containing glycine, and the greater increase when ATP is also present in the medium, probably accounts for the increased incorporation of the methylene carbon atom of glycine into fatty acids found previously under these conditions (Gajdos, Gajdos-Török, Gorchein, Neuberger & Tait, 1968). (biochemj.org)
  • high glycine
  • Many children with temporarily high glycine levels go on to develop normally and experience few long-term medical problems. (nih.gov)
  • As for the high glycine level, I also suspected NKH (non-ketotic hyperglycinemia) but my plasma amino acid level of it came back slightly low, but I will confirm that with a genetic test just to rule out the possibility of having NKH. (medhelp.org)
  • GlyT2 main physiological role is to recapture glycine released in the synaptic cleft and to maintain high glycine concentration in the presynaptic neuron. (wikipedia.org)
  • encephalopathy
  • Glycine encephalopathy is caused by the shortage of an enzyme that normally breaks down glycine in the body. (nih.gov)
  • The most common form of glycine encephalopathy , called the classical type, appears shortly after birth. (nih.gov)
  • Researchers have identified several other types of glycine encephalopathy with variable signs and symptoms. (nih.gov)
  • Other atypical types of glycine encephalopathy appear later in childhood or adulthood and cause a variety of medical problems that primarily affect the nervous system. (nih.gov)
  • Rarely, the characteristic features of classical glycine encephalopathy improve with time. (nih.gov)
  • The worldwide incidence of glycine encephalopathy is unknown. (nih.gov)
  • About 80 percent of cases of glycine encephalopathy result from mutations in the GLDC gene, while AMT mutations cause 10 percent to 15 percent of all cases. (nih.gov)
  • Applegarth DA, Toone JR. Glycine encephalopathy (nonketotic hyperglycinaemia) : review and update. (nih.gov)
  • Applegarth DA, Toone JR. Glycine encephalopathy (nonketotic hyperglycinemia): comments and speculations. (nih.gov)
  • Glycine encephalopathy (also known as non-ketotic hyperglycinemia or NKH) is a rare autosomal recessive disorder of glycine metabolism. (wikipedia.org)
  • After phenylketonuria, glycine encephalopathy is the second most common disorder of amino acid metabolism. (wikipedia.org)
  • Glycine encephalopathy is sometimes referred to as "nonketotic hyperglycinemia" (NKH), as a reference to the biochemical findings seen in patients with the disorder, and to distinguish it from the disorders that cause "ketotic hyperglycinemia" (seen in propionic acidemia and several other inherited metabolic disorders). (wikipedia.org)
  • To avoid confusion, the term "glycine encephalopathy" is often used, as this term more accurately describes the clinical symptoms of the disorder. (wikipedia.org)
  • Glycine encephalopathy has an estimated incidence of 1 in 60,000, making it the second most common disorder of amino acid metabolism, after phenylketonuria. (wikipedia.org)
  • Defects in three of these four genes have been linked to glycine encephalopathy. (wikipedia.org)
  • However, to date there have been no mutations in GCSL found to be associated with glycine encephalopathy. (wikipedia.org)
  • Defects in these subunits can cause glycine encephalopathy, although some causes of the disease are still unknown. (wikipedia.org)
  • All forms of glycine encephalopathy show elevated levels of glycine in the plasma, as well as in cerebral spinal fluid (CSF). (wikipedia.org)
  • Pathways
  • This conversion is readily reversible: CO2 + NH+ 4 + N5,N10-Methylene tetrahydrofolate + NADH + H+ ⇌ Glycine + tetrahydrofolate + NAD+ Glycine is degraded via three pathways. (wikipedia.org)
  • proteins
  • The principal function of glycine is as a precursor to proteins. (wikipedia.org)
  • Most proteins incorporate only small quantities of glycine, a notable exception being collagen, which contains about 35% glycine due to its periodically repeated role in the formation of collagen's helix structure in conjunction with hydroxyproline. (wikipedia.org)
  • GlyT2
  • Glycine transporter 2 (GlyT2) also known as the sodium- and chloride-dependent glycine transporter 2 or solute carrier family 6 member 5 is a protein that in humans is encoded by the SLC6A5 gene. (wikipedia.org)
  • Glycine uptake mediated by GlyT2 is electrogenic, coupled to three Na+ and one Cl− (i.e. two positive charges per glycine). (wikipedia.org)
  • Inactivation of GlyT2 in knockout mice is lethal during the second post-natal week as the absence of GlyT2 disrupts inhibitory transmission by reducing glycine release. (wikipedia.org)
  • The glycine transporter GlyT2 controls the dynamics of synaptic vesicle refilling in inhibitory spinal cord neurons. (wikipedia.org)
  • atypical
  • A number of small, randomized control trials have been done looking at the effect of adding glycine to atypical antipsychotics and the impact that would have on symptoms. (schizophrenia.com)
  • genes
  • The aptamers were originally thought to cooperatively bind glycine to regulate the expression of downstream genes. (wikipedia.org)
  • It is thought that when glycine is in excess it will bind to both aptamers to activate these genes and facilitate glycine degradation. (wikipedia.org)
  • synthesis
  • An enzyme that catalyzes the transfer of an amidine group from arginine to glycine during creatine synthesis. (dictionary.com)
  • Although glycine can be isolated from hydrolyzed protein, this is not used for industrial production, as it can be manufactured more conveniently by chemical synthesis. (wikipedia.org)
  • The two main processes are amination of chloroacetic acid with ammonia, giving glycine and ammonium chloride, and the Strecker amino acid synthesis, which is the main synthetic method in the United States and Japan. (wikipedia.org)
  • Glycine is also cogenerated as an impurity in the synthesis of EDTA, arising from reactions of the ammonia coproduct. (wikipedia.org)
  • Glycine is not essential to the human diet, as it is biosynthesized in the body from the amino acid serine, which is in turn derived from 3-phosphoglycerate, but the metabolic capacity for glycine biosynthesis does not satisfy the need for collagen synthesis. (wikipedia.org)
  • The actual reaction catalyzed by AGAT is the synthesis of guanidinoacetate from arginine and glycine, with ornithine as a byproduct. (wikipedia.org)
  • Arginine:glycine amidinotransferase (AGAT) catalyzes the first step of creatine synthesis, resulting in the formation of guanidinoacetate, which is a substrate for creatine formation. (wikipedia.org)
  • 1993
  • 1993). Glycine pindanica (Fabaceae: Phaseolae), a new species from west Kimberley, Western Australia. (wikipedia.org)
  • reuptake
  • They serve to terminate the signaling of glycine by mediating its reuptake from the synaptic cleft back into the presynaptic neurons. (wikipedia.org)
  • tissues
  • A lack of this enzyme allows excess glycine to build up in tissues and organs, particularly the brain, leading to serious medical problems. (nih.gov)
  • When the complex is unable to metabolize glycine properly, this causes excess glycine to build up to toxic levels in the body's organs and tissues. (wikipedia.org)
  • Mutations
  • Mutations in either the AMT or GLDC gene prevent the complex from breaking down glycine properly. (nih.gov)