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aspartic acid and Peroxisomal Disorders

aspartic acid has been researched along with Peroxisomal Disorders in 4 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.

Peroxisomal Disorders: A heterogeneous group of inherited metabolic disorders marked by absent or dysfunctional PEROXISOMES. Peroxisomal enzymatic abnormalities may be single or multiple. Biosynthetic peroxisomal pathways are compromised, including the ability to synthesize ether lipids and to oxidize long-chain fatty acid precursors. Diseases in this category include ZELLWEGER SYNDROME; INFANTILE REFSUM DISEASE; rhizomelic chondrodysplasia (CHONDRODYSPLASIA PUNCTATA, RHIZOMELIC); hyperpipecolic acidemia; neonatal adrenoleukodystrophy; and ADRENOLEUKODYSTROPHY (X-linked). Neurologic dysfunction is a prominent feature of most peroxisomal disorders.

Research Excerpts

ExcerptRelevanceReference
"Late juvenile neuronal ceroid lipofuscinosis (NCL) is a lysosomal neurodegenerative disorder caused by the accumulation of lipopigment in neurons."1.30MR imaging and localized proton MR spectroscopy in late infantile neuronal ceroid lipofuscinosis. ( Grodd, W; Klose, U; Nägele, T; Schwab, A; Seeger, U; Seitz, D, 1998)
"Despite regional variability of demyelination, proton magnetic resonance spectroscopy revealed a specific metabolic pattern in all patients, with only moderate reduction of N-acetylaspartate, normal or reduced choline-containing compounds, normal or enhanced myo-inositol and no detectable lactate, which differs from findings in progressive cerebral adrenoleukodystrophy which usually exhibits a severe reduction of N-acetylaspartate and marked increases of choline-containing compounds, myo-inositol, and lactate."1.29Arrested cerebral adrenoleukodystrophy: a clinical and proton magnetic resonance spectroscopy study in three patients. ( Frahm, J; Hanefeld, F; Hunneman, DH; Jost, W; Korenke, GC; Krasemann, E; Pouwels, PJ; Stoeckler, S, 1996)

Research

Studies (4)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's3 (75.00)18.2507
2000's1 (25.00)29.6817
2010's0 (0.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Korenke, GC1
Pouwels, PJ1
Frahm, J1
Hunneman, DH1
Stoeckler, S1
Krasemann, E1
Jost, W1
Hanefeld, F1
Seitz, D1
Grodd, W1
Schwab, A1
Seeger, U1
Klose, U1
Nägele, T1
Gärtner, J1
Preuss, N1
Brosius, U1
Biermanns, M1
Groenendaal, F1
Bianchi, MC1
Battini, R1
Tosetti, M1
Boldrini, A1
de Vries, LS1
Cioni, G1

Other Studies

4 other studies available for aspartic acid and Peroxisomal Disorders

ArticleYear
Arrested cerebral adrenoleukodystrophy: a clinical and proton magnetic resonance spectroscopy study in three patients.
    Pediatric neurology, 1996, Volume: 15, Issue:2

    Topics: Aspartic Acid; Brain; Brain Diseases, Metabolic; Child; Choline; Demyelinating Diseases; Female; Hum

1996
MR imaging and localized proton MR spectroscopy in late infantile neuronal ceroid lipofuscinosis.
    AJNR. American journal of neuroradiology, 1998, Volume: 19, Issue:7

    Topics: Aspartic Acid; Brain; Brain Diseases; Case-Control Studies; Cerebellum; Cerebral Ventricles; Child;

1998
Mutations in PEX1 in peroxisome biogenesis disorders: G843D and a mild clinical phenotype.
    Journal of inherited metabolic disease, 1999, Volume: 22, Issue:3

    Topics: Aspartic Acid; ATPases Associated with Diverse Cellular Activities; Child; Glycine; Humans; Infant;

1999
Proton magnetic resonance spectroscopy (1H-MRS) of the cerebrum in two young infants with Zellweger syndrome.
    Neuropediatrics, 2001, Volume: 32, Issue:1

    Topics: Aspartic Acid; Brain; Choline; Creatine; Diagnosis, Differential; Dominance, Cerebral; Energy Metabo

2001