Page last updated: 2024-11-08

aspartic acid and MELAS Syndrome

aspartic acid has been researched along with MELAS Syndrome in 11 studies

Aspartic Acid: One of the non-essential amino acids commonly occurring in the L-form. It is found in animals and plants, especially in sugar cane and sugar beets. It may be a neurotransmitter.
aspartic acid : An alpha-amino acid that consists of succinic acid bearing a single alpha-amino substituent
L-aspartic acid : The L-enantiomer of aspartic acid.

MELAS Syndrome: A mitochondrial disorder characterized by focal or generalized seizures, episodes of transient or persistent neurologic dysfunction resembling strokes, and ragged-red fibers on muscle biopsy. Affected individuals tend to be normal at birth through early childhood, then experience growth failure, episodic vomiting, and recurrent cerebral insults resulting in visual loss and hemiparesis. The cortical lesions tend to occur in the parietal and occipital lobes and are not associated with vascular occlusion. VASCULAR HEADACHE is frequently associated and the disorder tends to be familial. (From Joynt, Clinical Neurology, 1992, Ch56, p117)

Research Excerpts

ExcerptRelevanceReference
"To assess the utility of Magnetic Resonance Spectroscopy (MRS) as a biomarker of response to L-arginine in mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS)."3.91MELAS: Monitoring treatment with magnetic resonance spectroscopy. ( Chong, RA; DeGiorgio, CM; Galati, A; Hovsepian, DA; Mazumder, R; Mishra, S; Yim, C, 2019)
" Mutation carriers included 45 patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS); 11 participants who would develop the MELAS syndrome during follow-up (converters); and 79 participants who would not develop the MELAS syndrome during follow-up (nonconverters)."3.80Cerebral metabolic abnormalities in A3243G mitochondrial DNA mutation carriers. ( De Vivo, D; DiMauro, S; Engelstad, KM; Hinton, V; Kaufmann, P; Mao, X; Shungu, D; Weiduschat, N, 2014)
"To clarify the roles of serial MR spectroscopy (MRS) and continuous arterial spin labeling (CASL) perfusion images for evaluating cerebral lesions in patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS)."3.76Pathophysiologic evaluation of MELAS strokes by serially quantified MRS and CASL perfusion images. ( Ikawa, M; Kimura, H; Kudo, T; Kuriyama, M; Okazawa, H; Shimizu, Y; Toyooka, M; Tsujikawa, T; Uematsu, H; Yoneda, M, 2010)
"1H magnetic resonance spectroscopy (MRS) of the brain and (31)P MRS and saturation transfer of resting skeletal muscle were used to investigate intracellular metabolites and fluxes through the creatine kinase (CK) reaction in a patient with the syndrome of mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS)."3.71Application of NMR spectroscopy to monitoring MELAS treatment: a case report. ( Hilbich, T; Kurlemann, G; Möller, HE; Schuierer, G; Wiedermann, D, 2002)
"Localized brain proton MR spectra were acquired from patients with different mitochondrial encephalomyopathies (myoclonus epilepsy with ragged-red fibers [MERRF], Kearns-Sayre syndrome [KSS], and mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes [MELAS])."3.68Proton MR spectroscopic characterization of differences in regional brain metabolic abnormalities in mitochondrial encephalomyopathies. ( Andermann, F; Arnold, DL; Karpati, G; Mathews, PM; Silver, K, 1993)
"Six patients with MELAS (age 28 ± 13 years, 3 [50%] female) and 17 with MSS (age 45 ± 11 years, 7 [41%] female) and 39 sex- and age-matched healthy controls (HC) were enrolled."1.62Brain MRS correlates with mitochondrial dysfunction biomarkers in MELAS-associated mtDNA mutations. ( Amore, G; Bianchini, C; Caporali, L; Carelli, V; Cortesi, I; Di Vito, L; Evangelisti, S; Gramegna, LL; La Morgia, C; Liguori, R; Lodi, R; Manners, DN; Maresca, A; Talozzi, L; Testa, C; Tonon, C; Valentino, ML, 2021)
"The two patients with MELAS syndrome had large decreases of NAA signal (50% and 20% of normal values, respectively) from their occipital lobe lesions during the acute stroke-like episodes."1.29Reversible decreases in N-acetylaspartate after acute brain injury. ( Arnold, DL; De Stefano, N; Matthews, PM, 1995)

Research

Studies (11)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's2 (18.18)18.2507
2000's2 (18.18)29.6817
2010's5 (45.45)24.3611
2020's2 (18.18)2.80

Authors

AuthorsStudies
Fukuda, M1
Nagao, Y1
Gramegna, LL1
Evangelisti, S1
Di Vito, L1
La Morgia, C1
Maresca, A1
Caporali, L1
Amore, G1
Talozzi, L1
Bianchini, C1
Testa, C1
Manners, DN1
Cortesi, I1
Valentino, ML1
Liguori, R1
Carelli, V1
Tonon, C1
Lodi, R1
Wang, R1
Hu, B1
Sun, C1
Geng, D1
Lin, J1
Li, Y1
Hovsepian, DA1
Galati, A1
Chong, RA1
Mazumder, R1
DeGiorgio, CM1
Mishra, S1
Yim, C1
Weiduschat, N1
Kaufmann, P1
Mao, X1
Engelstad, KM1
Hinton, V1
DiMauro, S1
De Vivo, D1
Shungu, D1
Tsujikawa, T1
Yoneda, M1
Shimizu, Y1
Uematsu, H1
Toyooka, M1
Ikawa, M1
Kudo, T1
Okazawa, H1
Kuriyama, M1
Kimura, H1
Liu, Z1
Zheng, D1
Wang, X1
Zhang, J1
Xie, S1
Xiao, J1
Jiang, X1
Mathews, PM1
Andermann, F1
Silver, K1
Karpati, G1
Arnold, DL2
De Stefano, N1
Matthews, PM1
Kim, HS1
Kim, DI1
Lee, BI1
Jeong, EK1
Choi, C1
Lee, JD1
Yoon, PH1
Kim, EJ1
Kim, SH1
Yoon, YK1
Möller, HE1
Wiedermann, D1
Kurlemann, G1
Hilbich, T1
Schuierer, G1

Clinical Trials (2)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
Mitochondrial Encephalomyopathies and Mental Retardation: Investigations of Clinical Syndromes Associated With MtDNA Point Mutations[NCT01532791]300 participants (Anticipated)Observational2004-07-31Recruiting
Mapping the Natural History of Traumatic Spinal Cord Injury in the Sensorimotor Cortex Using Functional Magnetic Resonance Imaging, Magnetic Resonance Spectroscopy and Diffusion Tensor Imaging[NCT00790361]20 participants (Actual)Observational2009-06-18Completed
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Other Studies

11 other studies available for aspartic acid and MELAS Syndrome

ArticleYear
Dynamic derangement in amino acid profile during and after a stroke-like episode in adult-onset mitochondrial disease: a case report.
    Journal of medical case reports, 2019, Oct-21, Volume: 13, Issue:1

    Topics: Arginine; Aspartic Acid; Biomarkers; Deafness; Diabetes Mellitus, Type 2; DNA, Mitochondrial; Growth

2019
Brain MRS correlates with mitochondrial dysfunction biomarkers in MELAS-associated mtDNA mutations.
    Annals of clinical and translational neurology, 2021, Volume: 8, Issue:6

    Topics: Adolescent; Adult; Aged; Aspartic Acid; Biomarkers; Cerebellum; Cerebral Cortex; Choline; DNA, Mitoc

2021
Metabolic abnormality in acute stroke-like lesion and its relationship with focal cerebral blood flow in patients with MELAS: Evidence from proton MR spectroscopy and arterial spin labeling.
    Mitochondrion, 2021, Volume: 59

    Topics: Adolescent; Adult; Aspartic Acid; Brain; Case-Control Studies; Choline; Creatine; Female; Glutamic A

2021
MELAS: Monitoring treatment with magnetic resonance spectroscopy.
    Acta neurologica Scandinavica, 2019, Volume: 139, Issue:1

    Topics: Arginine; Aspartic Acid; Brain; Choline; Female; Humans; Magnetic Resonance Spectroscopy; MELAS Synd

2019
Cerebral metabolic abnormalities in A3243G mitochondrial DNA mutation carriers.
    Neurology, 2014, Mar-04, Volume: 82, Issue:9

    Topics: Acidosis, Lactic; Adult; Aged; Aspartic Acid; Cerebral Cortex; Choline; Creatine; DNA, Mitochondrial

2014
Pathophysiologic evaluation of MELAS strokes by serially quantified MRS and CASL perfusion images.
    Brain & development, 2010, Volume: 32, Issue:2

    Topics: Adult; Aspartic Acid; Brain; Cerebrovascular Circulation; Child; Diagnostic Imaging; Female; Humans;

2010
Apparent diffusion coefficients of metabolites in patients with MELAS using diffusion-weighted MR spectroscopy.
    AJNR. American journal of neuroradiology, 2011, Volume: 32, Issue:5

    Topics: Adolescent; Adult; Aspartic Acid; Brain; Child; Choline; Creatinine; Diffusion; Female; Humans; Magn

2011
Proton MR spectroscopic characterization of differences in regional brain metabolic abnormalities in mitochondrial encephalomyopathies.
    Neurology, 1993, Volume: 43, Issue:12

    Topics: Aspartic Acid; Brain; Choline; Creatine; Humans; Kearns-Sayre Syndrome; Lactates; Lactic Acid; Magne

1993
Reversible decreases in N-acetylaspartate after acute brain injury.
    Magnetic resonance in medicine, 1995, Volume: 34, Issue:5

    Topics: Acute Disease; Aspartic Acid; Brain; Demyelinating Diseases; Humans; Magnetic Resonance Spectroscopy

1995
Diffusion-weighted image and MR spectroscopic analysis of a case of MELAS with repeated attacks.
    Yonsei medical journal, 2001, Volume: 42, Issue:1

    Topics: Adolescent; Aspartic Acid; Brain; Creatine; Diffusion; Female; Humans; Magnetic Resonance Imaging; M

2001
Application of NMR spectroscopy to monitoring MELAS treatment: a case report.
    Muscle & nerve, 2002, Volume: 25, Issue:4

    Topics: Adolescent; Aspartic Acid; Brain; Creatine; Female; Glutamic Acid; Humans; Inositol; Lactic Acid; Ma

2002
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