Contact and support
Need help, have a question, or want to contact the ResearchHub team?
© 2026 ResearchHub. Built for responsible scholarly connection.
Privacy-aware community infrastructureResearch graph
References from The glycine N -acyltransferases, GLYAT and GLYATL1, contribute to the detoxification of isovaleryl-CoA - an in-silico and in vitro validation. Local targets link to admitted publications; unresolved targets remain external evidence.
Tandem mass spectrometric analysis for amino, organic, and fatty acid disorders in newborn dried blood spots: a two-year summary from the New England Newborn Screening Program
10.1093/clinchem/47.11.1945 · 2001 · External reference
Use of tandem mass spectrometry for multianalyte screening of dried blood specimens from newborns
10.1373/clinchem.2003.022178 · 2003 · External reference
A common mutation is associated with a mild, potentially asymptomatic phenotype in patients with isovaleric acidemia diagnosed by newborn screening
10.1086/426318 · 2004 · External reference
Newborn screening and disease variants predict neurological outcome in isovaleric aciduria
10.1002/jimd.12364 · 2021 · External reference
An overview of benefits and challenges of rare disease biobanking in Africa, Focusing on South Africa
10.1089/bio.2020.0108 · 2021 · External reference
Isovaleric acidemia: a new genetic defect of leucine metabolism
10.1073/pnas.56.1.236 · 1966 · External reference
Isovaleric acidemia: new aspects of genetic and phenotypic heterogeneity
2006 · External reference
The mitochondrial permeability transition: a new pathophysiological mechanism for Reye's syndrome and toxic liver injury
10.1016/s0022-3565(25)20745-5 · 1996 · External reference
Clinical and neurocognitive outcome in symptomatic isovaleric acidemia
10.1186/1750-1172-7-9 · 2012 · External reference
Isovaleric acidemia: new aspects of genetic and phenotypic heterogeneity
10.1002/ajmg.c.30089 · 2006 · External reference
A common mutation is associated with a mild, potentially asymptomatic phenotype in patients with isovaleric acidemia diagnosed by newborn screening
10.1086/426318 · 2004 · External reference
10.3390/ijns4010007
10.3390/ijns4010007 · External reference
Clinical variability of isovaleric acidemia in a genetically homogeneous population
2012 · External reference
Induction of oxidative stress by the metabolites accumulating in isovaleric acidemia in brain cortex of young rats
10.1080/10715760802311179 · 2008 · External reference
Isovaleric acid reduces Na+, K+-ATPase activity in synaptic membranes from cerebral cortex of young rats
10.1007/s10571-007-9143-3 · 2007 · External reference
Inhibition of N-acetylglutamate synthase by various monocarboxylic and dicarboxylic short-chain coenzyme A esters and the production of alternative glutamate esters
10.1016/j.bbadis.2013.04.027 · 2014 · External reference
N-carbamylglutamate treatment for acute neonatal hyperammonemia in isovaleric acidemia
10.1007/s00431-010-1362-9 · 2011 · External reference
Long term follow-up of polish patients with isovaleric aciduria. Clinical and molecular delineation of isovaleric aciduria
10.3390/diagnostics10100738 · 2020 · External reference
Treatment of isovaleric acidemia with glycine supplements
10.1203/00006450-198704010-00753 · 1987 · External reference
Effect of carnitine administration on glycine metabolism in patients with isovaleric acidemia: significance of acetylcarnitine determination to estimate the proper carnitine dose
10.1620/tjem.179.101 · 1996 · External reference
The isolation and identification of N-isovalerylglycine from urine of patients with isovaleric acidemia
10.1016/s0021-9258(18)99599-2 · 1967 · External reference
Unresolved reference
2013 · External reference
The isolation and identification of N-isovalerylglycine from urine of patients with isovaleric acidemia
1967 · External reference
Unresolved reference
2001 · External reference
Single amino acid supplementation in aminoacidopathies: a systematic review
10.1186/1750-1172-9-7 · 2014 · External reference
Isovaleric acidemia: Therapeutic response to supplementation with glycine, l-carnitine, or both in combination and a 10-year follow-up case study
2017 · External reference
Idiopathic hyperglycinemia and hyperglycinuria: a new disorder of amino acid metabolism. 1
10.1542/peds.27.4.522 · 1961 · External reference
The response to L-carnitine and glycine therapy in isovaleric acidaemia
10.1007/bf00441737 · 1986 · External reference
Therapeutic effects of glycine in isovaleric acidemia
10.1203/00006450-197601000-00005 · 1976 · External reference
Glycine N-acylase: purification and properties
1954 · External reference
The treatment of isovaleric acidemia with glycine supplement
10.1203/00006450-198807000-00004 · 1988 · External reference
The specificity of glycine-N-acylase and acylglycine excretion in the organicacidaemias
1974 · External reference
Benzoic acid and its derivatives as naturally occurring compounds in foods and as additives: Uses, exposure, and controversy
10.1080/10408398.2015.1087964 · 2017 · External reference
10.1007/s00438-018-1460-3
10.1007/s00438-018-1460-3 · External reference
The metabolic fate of dietary polyphenols in humans
10.1016/s0891-5849(02)00877-8 · 2002 · External reference
10.1016/j.biochi.2015.08.002
10.1016/j.biochi.2015.08.002 · External reference
Evidence for a medium-chain fatty acid: coenzyme A ligase (adenosine monophosphate) that activates salicylate
10.1016/s0026-895x(25)12663-1 · 1971 · External reference
Role of hepatic fatty acid:coenzyme A ligases in the metabolism of xenobiotic carboxylic acids
10.1111/j.1440-1681.1998.tb02152.x · 1998 · External reference
Benzoyl-coenzyme A:glycine N-acyltransferase and phenylacetyl-coenzyme A:glycine N-acyltransferase from bovine liver mitochondria. Purification and characterization
1979 · External reference
10.1016/j.ymgme.2007.12.005
10.1016/j.ymgme.2007.12.005 · External reference
Synthesis of phenylacetylglutamine by human tissue
10.1016/s0021-9258(18)70632-7 · 1957 · External reference
Acyl-CoA: glycine N-acyltransferase: in vitro studies on the glycine conjugation of straight- and branched-chained acyl-CoA esters in human liver
1986 · External reference
Characterization of the acyl-CoA: Amino acid N-acyltransferases from primate liver mitochondria
10.1002/jbt.2570090307 · 1994 · External reference
10.1073/pnas.1919250117
10.1073/pnas.1919250117 · External reference
10.1093/brain/awt328
10.1093/brain/awt328 · External reference
Health consequences of catabolic synthesis of hippuric acid in humans
10.2174/157488410790410588 · 2010 · External reference
Molecular cloning and characterization of a novel human glycine-n-acyltransferase gene GLYATL1, which activates transcriptional activity of HSE pathway
10.3390/i8050433 · 2007 · External reference
Designation of enzyme activity of glycine-N-acyltransferase family genes and depression of glycine-N-acyltransferase in human hepatocellular carcinoma
2012 · External reference
New insights into the catalytic mechanism of human glycine N-acyltransferase
10.1002/jbt.21963 · 2017 · External reference
Enzymatic characterization and elucidation of the catalytic mechanism of a recombinant bovine glycine N-acyltransferase
10.1124/dmd.111.041657 · 2012 · External reference
10.1016/j.pisc.2014.12.003
10.1016/j.pisc.2014.12.003 · External reference
Functional characterisation of three glycine n-acyltransferase variants and the effect on glycine conjugation to Benzoyl-CoA
10.3390/ijms22063129 · 2021 · External reference
10.1093/bioinformatics/16.4.404
10.1093/bioinformatics/16.4.404 · External reference
10.1093/bioinformatics/btu744
10.1093/bioinformatics/btu744 · External reference
10.1038/cr.2009.87
10.1038/cr.2009.87 · External reference
10.1093/nar/gkg520
10.1093/nar/gkg520 · External reference
10.1093/nar/gky427
10.1093/nar/gky427 · External reference
10.1093/nar/gku340
10.1093/nar/gku340 · External reference
10.1107/s0021889892009944
10.1107/s0021889892009944 · External reference
10.1126/science.1853201
10.1126/science.1853201 · External reference
10.1038/356083a0
10.1038/356083a0 · External reference
Homology modeling and protein engineering of alkane monooxygenase in Burkholderia thailandensis MSMB121: in silico insights
10.1007/s00894-014-2340-3 · 2014 · External reference
10.1002/pro.5560020916
10.1002/pro.5560020916 · External reference
Homology modeling and virtual screening approaches to identify potent inhibitors of VEB-1 β-lactamase
10.1186/1742-4682-10-22 · 2013 · External reference
10.1093/nar/gkm290
10.1093/nar/gkm290 · External reference
10.1002/j.1460-2075.1986.tb04288.x
10.1002/j.1460-2075.1986.tb04288.x · External reference
10.1016/j.bpj.2011.10.024
10.1016/j.bpj.2011.10.024 · External reference
10.1002/jcc.21334
10.1002/jcc.21334 · External reference
Purification to homogeneity of mitochondrial acyl coa:glycine n-acyltransferase from human liver
10.1006/bbrc.1994.2817 · 1994 · External reference
Benzoyl coenzyme A and hippurate synthesis
10.1016/s0021-9258(19)52362-6 · 1953 · External reference
10.1093/nar/gkv315
10.1093/nar/gkv315 · External reference
Unresolved reference
External reference
Characterisation of the influence of genetic variations on the enzyme activity of a recombinant human glycine N-acyltransferase
10.1016/j.gene.2012.12.003 · 2013 · External reference
10.1021/acs.accounts.5b00516
10.1021/acs.accounts.5b00516 · External reference
The utilization of alanine, glutamic acid, and serine as amino acid substrates for glycine N-acyltransferase
2000 · External reference
Purification to homogeneity of mitochondrial acyl coa:glycine n-acyltransferase from human liver
1994 · External reference
Frequent sequence variants of human glycine N-acyltransferase (GLYAT) and inborn errors of metabolism
2021 · External reference
Pharmacokinetics of salicylate elimination in man
10.1002/jps.2600540703 · 1965 · External reference
The effect of liver injury on the conjugation of benzoic acid in the dog
10.1016/s0021-9258(18)76072-9 · 1932 · External reference
The chemical estimation of acyl glucuronides and its application to studies on the metabolism of benzoate and salicylate in man
10.1172/jci103424 · 1957 · External reference
Excretion of N-isovalerylglutamic acid in isovaleric acidemia
10.1016/0009-8981(81)90030-9 · 1981 · External reference
Organic acid profile of isovaleric acidemia: a comprehensive metabolomics approach
10.1007/s11306-013-0501-5 · 2013 · External reference
Identification of 19 new metabolites induced by abnormal amino acid conjugation in isovaleric acidemia
10.1373/clinchem.2005.048421 · 2005 · External reference
Isovaleric acidemia: response to a leucine load after three weeks of supplementation with glycine, L-carnitine, and combined glycine-carnitine therapy
10.1016/s0022-3476(96)70081-1 · 1996 · External reference
Isovaleric acidemia: use of glycine therapy in neonates
10.1056/nejm197811022991807 · 1978 · External reference
Glycine therapy in isovaleric acidemia
10.1016/s0022-3476(78)80164-4 · 1978 · External reference
Expression, purification, and characterization of mouse glycine N-acyltransferase in Escherichia coli
10.1016/j.pep.2014.02.007 · 2014 · External reference
Unresolved reference
2001 · External reference
Unresolved reference
2010 · External reference
Dependence of glycine conjugation on availability of glycine: role of the glycine cleavage system
10.3109/00498259309059370 · 1993 · External reference
10.1517/17425255.2013.796929
10.1517/17425255.2013.796929 · External reference
Glycine cleavage system: reaction mechanism, physiological significance, and hyperglycinemia
10.2183/pjab.84.246 · 2008 · External reference
Allosteric control of substrate specificity of the escherichia coli ADP-glucose pyrophosphorylase
10.3389/fchem.2017.00041 · 2017 · External reference
10.3109/03602532.2014.908903
10.3109/03602532.2014.908903 · External reference
The concentrations of free amino acids in human liver tissue obtained during laparoscopic surgery
10.1111/j.1475-097x.1996.tb00570.x · 1996 · External reference
Quantitative modelling of amino acid transport and homeostasis in mammalian cells
10.1038/s41467-021-25563-x · 2021 · External reference
High-dose intravenous infusion of irrigating fluids containing glycine and mannitol in the pig
10.1006/jsre.2000.6028 · 2001 · External reference
10.1093/jn/134.6.1633s
10.1093/jn/134.6.1633s · External reference
Isovaleric acidemia: medical and neurodevelopmental effects of long-term therapy
10.1016/s0022-3476(88)80528-6 · 1988 · External reference
Long-term neurological outcome of a cohort of 80 patients with classical organic acidurias
10.1186/1750-1172-8-148 · 2013 · External reference
Changing plasma and urinary organic acid levels in a patient with isovaleric acidemia during an attack
10.1203/00006450-198209000-00013 · 1982 · External reference
Treatment of isovaleric acidemia with glycine supplements
10.1203/00006450-198704010-00753 · 1987 · External reference
Isovaleric acidemia: response to a leucine load after three weeks of supplementation with glycine, L-carnitine, and combined glycine-carnitine therapy
10.1016/s0022-3476(96)70081-1 · 1996 · External reference
