Page last updated: 2024-10-19

pipecolic acid and Aura

pipecolic acid has been researched along with Aura in 18 studies

pipecolic acid: RN given refers to cpd without isomeric designation
pipecolic acid : A piperidinemonocarboxylic acid in which the carboxy group is located at position C-2.
pipecolate : A piperidinecarboxylate that is the conjugate base of pipecolic acid.

Research Excerpts

ExcerptRelevanceReference
"Pyridoxine-dependent epilepsy (PDE) is a recessive genetic disease characterized by epileptic encephalopathy with therapeutic response to pharmacological doses of pyridoxine and resistance to anti-epileptic treatments."8.02Clinical and biochemical outcome of a patient with pyridoxine-dependent epilepsy treated by triple therapy (pyridoxine supplementation, lysine-restricted diet, and arginine supplementation). ( Benoist, JF; Mention, K; Minet, P; Miret, A; Remerand, G; Sarret, C, 2021)
"Using our laboratory database, we identified patients with vitamin B6-dependent epilepsy and extracted their data on the concentrations of pyridoxal 5'-phosphate, pyridoxal, pipecolic acid, α-aminoadipic semialdehyde, and monoamine neurotransmitters."7.96Pyridoxal in the Cerebrospinal Fluid May Be a Better Indicator of Vitamin B6-dependent Epilepsy Than Pyridoxal 5'-Phosphate. ( Akiyama, T; Baba, S; Dowa, Y; Fukuyama, T; Hamano, SI; Hasegawa, K; Hyodo, Y; Imai, K; Ishihara, N; Kobayashi, K; Koike, T; Kubota, M; Oboshi, T; Okanishi, T; Shibasaki, J; Shimbo, H; Shiraku, H; Takano, K; Takeshita, S; Yamamoto, T, 2020)
"The measurements of lysine metabolites provide valuable information for the rapid diagnosis of pyridoxine-dependent epilepsy (PDE)."7.91Simultaneous quantification of alpha-aminoadipic semialdehyde, piperideine-6-carboxylate, pipecolic acid and alpha-aminoadipic acid in pyridoxine-dependent epilepsy. ( Gong, P; Jiang, S; Jiang, Y; Li, H; Wang, J; Wu, M; Wu, Y; Xue, J; Yang, W; Yang, Z; Yuzyuk, T; Zhang, Y, 2019)
"Pyridoxine-Dependent Epilepsy (PDE) is a recessive disorder caused by deficiency of α-aminoadipic semialdehyde dehydrogenase in the catabolic pathway of lysine."7.83Effect of dietary lysine restriction and arginine supplementation in two patients with pyridoxine-dependent epilepsy. ( Botto, LD; De Biase, I; Liu, A; Longo, N; Pasquali, M; Thomas, A; Viau, K; Yuzyuk, T, 2016)
"To analyze the clinical and genetic characteristics of patients with pyridoxine dependent epilepsy (PDE), and build a method to detect and analyze the concentration of urinary pipecolic acid in PDE patients receiving pyridoxine treatment."7.83[Clinical and genetic characteristics and detection of urinary pipecolic acid in pyridoxine dependent epilepsy]. ( Jiang, YW; Li, H; Liu, XY; Qian, P; Wu, Y; Xue, J; Yang, ZX, 2016)
"To evaluate the efficacy and safety of dietary lysine restriction as an adjunct to pyridoxine therapy on biochemical parameters, seizure control, and developmental/cognitive outcomes in children with pyridoxine-dependent epilepsy (PDE) caused by antiquitin (ATQ) deficiency."7.78Lysine restricted diet for pyridoxine-dependent epilepsy: first evidence and future trials. ( Bok, LA; Cheng, B; Collet, JP; Connolly, M; Coughlin, CR; Das, AM; Gospe, SM; Hartmann, H; Jaggumantri, S; Jakobs, C; Mercimek-Mahmutoglu, S; Meyer, U; Plecko, BR; Sinclair, G; Stockler, S; Struys, E; van der Lee, JH; Van Hove, J; van Karnebeek, CD, 2012)
"Pyridoxine-dependent epilepsy, although described some decades ago, may still be an underdiagnosed disorder."7.73Pipecolic acid as a diagnostic marker of pyridoxine-dependent epilepsy. ( Baumeister, F; Baumgartner, M; Erwa, W; Hikel, C; Jakobs, C; Korenke, GC; Plecko, B; Schmitt, B; Stöckler-Ipsiroglu, S; Struys, E, 2005)
"Diagnosis of pyridoxine-dependent epilepsy is based on the clinical response to high-dosage application of pyridoxine."7.70Pipecolic acid elevation in plasma and cerebrospinal fluid of two patients with pyridoxine-dependent epilepsy. ( Erwa, W; Jakobs, C; Paschke, E; Plecko, B; Stöckler-Ipsiroglu, S; Struys, EA, 2000)
"Tiagabine has comparable anticonvulsant action to carbamazepine in rats and has anticonvulsant effects in non-human primates supporting the potential use of inhibitors of GABA uptake as therapy for epilepsy."5.29The gamma-aminobutyric acid uptake inhibitor, tiagabine, is anticonvulsant in two animal models of reflex epilepsy. ( Chapman, AG; Meldrum, BS; Parvez, NS; Smith, SE, 1995)
"Pyridoxine-dependent epilepsy (PDE) is a recessive genetic disease characterized by epileptic encephalopathy with therapeutic response to pharmacological doses of pyridoxine and resistance to anti-epileptic treatments."4.02Clinical and biochemical outcome of a patient with pyridoxine-dependent epilepsy treated by triple therapy (pyridoxine supplementation, lysine-restricted diet, and arginine supplementation). ( Benoist, JF; Mention, K; Minet, P; Miret, A; Remerand, G; Sarret, C, 2021)
"Using our laboratory database, we identified patients with vitamin B6-dependent epilepsy and extracted their data on the concentrations of pyridoxal 5'-phosphate, pyridoxal, pipecolic acid, α-aminoadipic semialdehyde, and monoamine neurotransmitters."3.96Pyridoxal in the Cerebrospinal Fluid May Be a Better Indicator of Vitamin B6-dependent Epilepsy Than Pyridoxal 5'-Phosphate. ( Akiyama, T; Baba, S; Dowa, Y; Fukuyama, T; Hamano, SI; Hasegawa, K; Hyodo, Y; Imai, K; Ishihara, N; Kobayashi, K; Koike, T; Kubota, M; Oboshi, T; Okanishi, T; Shibasaki, J; Shimbo, H; Shiraku, H; Takano, K; Takeshita, S; Yamamoto, T, 2020)
"The measurements of lysine metabolites provide valuable information for the rapid diagnosis of pyridoxine-dependent epilepsy (PDE)."3.91Simultaneous quantification of alpha-aminoadipic semialdehyde, piperideine-6-carboxylate, pipecolic acid and alpha-aminoadipic acid in pyridoxine-dependent epilepsy. ( Gong, P; Jiang, S; Jiang, Y; Li, H; Wang, J; Wu, M; Wu, Y; Xue, J; Yang, W; Yang, Z; Yuzyuk, T; Zhang, Y, 2019)
"Pyridoxine-Dependent Epilepsy (PDE) is a recessive disorder caused by deficiency of α-aminoadipic semialdehyde dehydrogenase in the catabolic pathway of lysine."3.83Effect of dietary lysine restriction and arginine supplementation in two patients with pyridoxine-dependent epilepsy. ( Botto, LD; De Biase, I; Liu, A; Longo, N; Pasquali, M; Thomas, A; Viau, K; Yuzyuk, T, 2016)
"To analyze the clinical and genetic characteristics of patients with pyridoxine dependent epilepsy (PDE), and build a method to detect and analyze the concentration of urinary pipecolic acid in PDE patients receiving pyridoxine treatment."3.83[Clinical and genetic characteristics and detection of urinary pipecolic acid in pyridoxine dependent epilepsy]. ( Jiang, YW; Li, H; Liu, XY; Qian, P; Wu, Y; Xue, J; Yang, ZX, 2016)
"Pipecolic acid (PA) is an important biochemical marker for the diagnosis of peroxisomal disorders."3.81Determination of plasma pipecolic acid by an easy and rapid liquid chromatography-tandem mass spectrometry method. ( Barraco, GM; Boenzi, S; Catesini, G; Dionisi-Vici, C; Iacovone, F; Inglese, R; Manco, M; Muraca, M; Rizzo, C; Semeraro, M, 2015)
"To evaluate the efficacy and safety of dietary lysine restriction as an adjunct to pyridoxine therapy on biochemical parameters, seizure control, and developmental/cognitive outcomes in children with pyridoxine-dependent epilepsy (PDE) caused by antiquitin (ATQ) deficiency."3.78Lysine restricted diet for pyridoxine-dependent epilepsy: first evidence and future trials. ( Bok, LA; Cheng, B; Collet, JP; Connolly, M; Coughlin, CR; Das, AM; Gospe, SM; Hartmann, H; Jaggumantri, S; Jakobs, C; Mercimek-Mahmutoglu, S; Meyer, U; Plecko, BR; Sinclair, G; Stockler, S; Struys, E; van der Lee, JH; Van Hove, J; van Karnebeek, CD, 2012)
"Pyridoxine-dependent epilepsy, although described some decades ago, may still be an underdiagnosed disorder."3.73Pipecolic acid as a diagnostic marker of pyridoxine-dependent epilepsy. ( Baumeister, F; Baumgartner, M; Erwa, W; Hikel, C; Jakobs, C; Korenke, GC; Plecko, B; Schmitt, B; Stöckler-Ipsiroglu, S; Struys, E, 2005)
"Elevated concentrations of pipecolic acid have been reported in plasma and CSF of patients with pyridoxine-dependent epilepsy, but its molecular background is unclear."3.73Pipecolic acid concentrations in brain tissue of nutritionally pyridoxine-deficient rats. ( Hoeger, H; Jakobs, C; Leschnik, M; Muehl, A; Plecko, B; Stoeckler-Ipsiroglu, S; Stromberger, C; Struys, E, 2005)
"Diagnosis of pyridoxine-dependent epilepsy is based on the clinical response to high-dosage application of pyridoxine."3.70Pipecolic acid elevation in plasma and cerebrospinal fluid of two patients with pyridoxine-dependent epilepsy. ( Erwa, W; Jakobs, C; Paschke, E; Plecko, B; Stöckler-Ipsiroglu, S; Struys, EA, 2000)
"Tiagabine has comparable anticonvulsant action to carbamazepine in rats and has anticonvulsant effects in non-human primates supporting the potential use of inhibitors of GABA uptake as therapy for epilepsy."1.29The gamma-aminobutyric acid uptake inhibitor, tiagabine, is anticonvulsant in two animal models of reflex epilepsy. ( Chapman, AG; Meldrum, BS; Parvez, NS; Smith, SE, 1995)

Research

Studies (18)

TimeframeStudies, this research(%)All Research%
pre-19901 (5.56)18.7374
1990's2 (11.11)18.2507
2000's4 (22.22)29.6817
2010's8 (44.44)24.3611
2020's3 (16.67)2.80

Authors

AuthorsStudies
Smith, SE1
Parvez, NS1
Chapman, AG1
Meldrum, BS1
Pfeiffer, M1
Draguhn, A1
Meierkord, H1
Heinemann, U1
Mathis, D3
Beese, K1
Rüegg, C1
Plecko, B6
Hersberger, M1
Akiyama, T1
Hyodo, Y1
Hasegawa, K1
Oboshi, T1
Imai, K1
Ishihara, N1
Dowa, Y1
Koike, T1
Yamamoto, T1
Shibasaki, J1
Shimbo, H1
Fukuyama, T1
Takano, K1
Shiraku, H1
Takeshita, S1
Okanishi, T1
Baba, S1
Kubota, M1
Hamano, SI1
Kobayashi, K1
Minet, P1
Sarret, C1
Miret, A1
Mention, K1
Benoist, JF1
Remerand, G1
Srinivasaraghavan, R1
Parameswaran, N1
Bürer, C1
Crowther, LM1
Poms, M1
Xue, J2
Wang, J1
Gong, P1
Wu, M1
Yang, W1
Jiang, S1
Wu, Y2
Jiang, Y1
Zhang, Y1
Yuzyuk, T2
Li, H2
Yang, Z1
Semeraro, M1
Muraca, M1
Catesini, G1
Inglese, R1
Iacovone, F1
Barraco, GM1
Manco, M1
Boenzi, S1
Dionisi-Vici, C1
Rizzo, C1
Leganés-Ramos, A1
Álvaro-Alonso, EA1
Martín de Rosales-Cabrera, AM1
Pérez-Encinas, M1
Thomas, A1
Viau, K1
Liu, A1
De Biase, I1
Botto, LD1
Pasquali, M1
Longo, N1
Yang, ZX1
Qian, P1
Jiang, YW1
Liu, XY1
van Karnebeek, CD1
Hartmann, H1
Jaggumantri, S1
Bok, LA1
Cheng, B1
Connolly, M1
Coughlin, CR1
Das, AM1
Gospe, SM1
Jakobs, C5
van der Lee, JH1
Mercimek-Mahmutoglu, S1
Meyer, U1
Struys, E3
Sinclair, G1
Van Hove, J1
Collet, JP1
Plecko, BR1
Stockler, S1
Hikel, C1
Korenke, GC1
Schmitt, B1
Baumgartner, M1
Baumeister, F1
Erwa, W2
Stöckler-Ipsiroglu, S2
Hoeger, H1
Stromberger, C1
Leschnik, M1
Muehl, A1
Stoeckler-Ipsiroglu, S1
Willemsen, MA1
Mavinkurve-Groothuis, AM1
Wevers, RA1
Rotteveel, JJ1
Paschke, E1
Struys, EA1
Barth, PG1
Schutgens, RB1
Bakkeren, JA1
Dingemans, KP1
Heymans, HS1
Douwes, AC1
van der Klei-van Moorsel, JM1

Clinical Trials (1)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
Neurodevelopmental Outcome of Early Dietary Lysine Restriction in Pyridoxine[NCT01795170]0 participants (Actual)Observational2013-04-30Withdrawn (stopped due to The study couldn't be initiated as we did not secure funding.)
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Other Studies

18 other studies available for pipecolic acid and Aura

ArticleYear
The gamma-aminobutyric acid uptake inhibitor, tiagabine, is anticonvulsant in two animal models of reflex epilepsy.
    European journal of pharmacology, 1995, Feb-06, Volume: 273, Issue:3

    Topics: Acoustic Stimulation; Animals; Anticonvulsants; Behavior, Animal; Carbamazepine; Epilepsy; Female; G

1995
Effects of gamma-aminobutyric acid (GABA) agonists and GABA uptake inhibitors on pharmacosensitive and pharmacoresistant epileptiform activity in vitro.
    British journal of pharmacology, 1996, Volume: 119, Issue:3

    Topics: Animals; Baclofen; Dose-Response Relationship, Drug; Entorhinal Cortex; Epilepsy; Female; GABA Agoni

1996
LC-MS/MS method for the differential diagnosis of treatable early onset inherited metabolic epilepsies.
    Journal of inherited metabolic disease, 2020, Volume: 43, Issue:5

    Topics: Aldehyde Dehydrogenase; Biomarkers; Chromatography, Liquid; Diagnosis, Differential; Epilepsy; Human

2020
Pyridoxal in the Cerebrospinal Fluid May Be a Better Indicator of Vitamin B6-dependent Epilepsy Than Pyridoxal 5'-Phosphate.
    Pediatric neurology, 2020, Volume: 113

    Topics: 5-Hydroxytryptophan; Adolescent; Child; Child, Preschool; Epilepsy; Female; Humans; Infant; Infant,

2020
Clinical and biochemical outcome of a patient with pyridoxine-dependent epilepsy treated by triple therapy (pyridoxine supplementation, lysine-restricted diet, and arginine supplementation).
    Acta neurologica Belgica, 2021, Volume: 121, Issue:6

    Topics: Arginine; Biomarkers; Child, Preschool; Dietary Supplements; Epilepsy; Female; Humans; Lysine; Pipec

2021
Antiquitin Deficiency with Adolescent Onset Epilepsy: Molecular Diagnosis in a Mother of Affected Offsprings.
    Neuropediatrics, 2018, Volume: 49, Issue:2

    Topics: Age of Onset; Aldehyde Dehydrogenase; Epilepsy; Humans; Magnetic Resonance Imaging; Male; Metabolic

2018
New insights into human lysine degradation pathways with relevance to pyridoxine-dependent epilepsy due to antiquitin deficiency.
    Journal of inherited metabolic disease, 2019, Volume: 42, Issue:4

    Topics: 2-Aminoadipic Acid; Aldehyde Dehydrogenase; Epilepsy; Humans; Lysine; Metabolic Networks and Pathway

2019
Simultaneous quantification of alpha-aminoadipic semialdehyde, piperideine-6-carboxylate, pipecolic acid and alpha-aminoadipic acid in pyridoxine-dependent epilepsy.
    Scientific reports, 2019, 08-06, Volume: 9, Issue:1

    Topics: 2-Aminoadipic Acid; Biomarkers; Child; Child, Preschool; Chromatography, Liquid; Epilepsy; Female; H

2019
Determination of plasma pipecolic acid by an easy and rapid liquid chromatography-tandem mass spectrometry method.
    Clinica chimica acta; international journal of clinical chemistry, 2015, Feb-02, Volume: 440

    Topics: Adolescent; Adult; Biomarkers; Calibration; Child; Child, Preschool; Chromatography, High Pressure L

2015
Oral formulation of pyridoxine for the treatment of pyridoxinedependent epilepsy in a paediatric patient.
    Farmacia hospitalaria : organo oficial de expresion cientifica de la Sociedad Espanola de Farmacia Hospitalaria, 2016, Mar-01, Volume: 40, Issue:2

    Topics: Drug Compounding; Epilepsy; Female; Humans; Hyperlysinemias; Infant, Newborn; Pipecolic Acids; Pyrid

2016
Effect of dietary lysine restriction and arginine supplementation in two patients with pyridoxine-dependent epilepsy.
    Molecular genetics and metabolism, 2016, Volume: 118, Issue:3

    Topics: Arginine; Biomarkers; Child, Preschool; Dietary Supplements; Epilepsy; Female; Humans; Infant; Lysin

2016
[Clinical and genetic characteristics and detection of urinary pipecolic acid in pyridoxine dependent epilepsy].
    Zhonghua er ke za zhi = Chinese journal of pediatrics, 2016, Volume: 54, Issue:8

    Topics: Aldehyde Dehydrogenase; Asian People; Child; Child, Preschool; Delayed Diagnosis; DNA Mutational Ana

2016
Lysine restricted diet for pyridoxine-dependent epilepsy: first evidence and future trials.
    Molecular genetics and metabolism, 2012, Volume: 107, Issue:3

    Topics: 2-Aminoadipic Acid; Aldehyde Dehydrogenase; Child; Child, Preschool; Cognition; Diet; Epilepsy; Fema

2012
Pipecolic acid as a diagnostic marker of pyridoxine-dependent epilepsy.
    Neuropediatrics, 2005, Volume: 36, Issue:3

    Topics: Anticonvulsants; Biomarkers; Brain; Epilepsy; Humans; Infant; Infant, Newborn; Pipecolic Acids; Pyri

2005
Pipecolic acid concentrations in brain tissue of nutritionally pyridoxine-deficient rats.
    Journal of inherited metabolic disease, 2005, Volume: 28, Issue:5

    Topics: Animal Nutritional Physiological Phenomena; Animals; Brain; Disease Models, Animal; Epilepsy; Female

2005
Pipecolic acid: a diagnostic marker in pyridoxine-dependent epilepsy.
    Annals of neurology, 2005, Volume: 58, Issue:4

    Topics: Child; Electroencephalography; Epilepsy; Follow-Up Studies; Humans; Male; Pipecolic Acids; Pyridoxin

2005
Pipecolic acid elevation in plasma and cerebrospinal fluid of two patients with pyridoxine-dependent epilepsy.
    Annals of neurology, 2000, Volume: 48, Issue:1

    Topics: 2-Aminoadipic Acid; Child; Epilepsy; Humans; Infant, Newborn; Male; Picolinic Acids; Pipecolic Acids

2000
A milder variant of Zellweger syndrome.
    European journal of pediatrics, 1985, Volume: 144, Issue:4

    Topics: Abnormalities, Multiple; Biopsy; Child, Preschool; Choroid; Epilepsy; Facial Bones; Genes, Recessive

1985