gamma-aminobutyric acid has been researched along with Neuronal Ceroid-Lipofuscinoses in 11 studies
gamma-Aminobutyric Acid: The most common inhibitory neurotransmitter in the central nervous system.
gamma-aminobutyric acid : A gamma-amino acid that is butanoic acid with the amino substituent located at C-4.
Neuronal Ceroid-Lipofuscinoses: A group of severe neurodegenerative diseases characterized by intracellular accumulation of autofluorescent wax-like lipid materials (CEROID; LIPOFUSCIN) in neurons. There are several subtypes based on mutations of the various genes, time of disease onset, and severity of the neurological defects such as progressive DEMENTIA; SEIZURES; and visual failure.
| Excerpt | Relevance | Reference |
|---|---|---|
| "Animal models of Batten disease and other neuronal storage disorders offer important opportunities to study the pathogenesis of brain dysfunction in this family of diseases." | 2.39 | Pathogenesis of brain dysfunction in Batten disease. ( Jolly, RD; March, PA; Schroeder, CE; Walkley, SU; Wurzelmann, S, 1995) |
| "Infantile Neuronal Ceroid Lipofuscinosis (INCL) results from mutations in the palmitoyl protein thioesterase (PPT1, CLN1) gene and is characterized by dramatic death of cortical neurons." | 1.33 | Mice with Ppt1Deltaex4 mutation replicate the INCL phenotype and show an inflammation-associated loss of interneurons. ( Fabritius, AL; Gentile, M; Jalanko, A; Kopra, O; Manninen, T; Minye, H; Peltonen, L; Rapola, J; Salonen, T; Vesa, J; von Schantz, C, 2005) |
| "Infantile neuronal ceroid lipofuscinosis (INCL) is one of a group of fatal hereditary lysosomal storage disorders." | 1.32 | Regional and cellular neuropathology in the palmitoyl protein thioesterase-1 null mutant mouse model of infantile neuronal ceroid lipofuscinosis. ( Bible, E; Cooper, JD; Gupta, P; Hofmann, SL, 2004) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 2 (18.18) | 18.2507 |
| 2000's | 8 (72.73) | 29.6817 |
| 2010's | 0 (0.00) | 24.3611 |
| 2020's | 1 (9.09) | 2.80 |
| Authors | Studies |
|---|---|
| Dang Do, AN | 1 |
| Baker, EH | 1 |
| Farmer, CA | 1 |
| Soldatos, AG | 1 |
| Thurm, AE | 1 |
| Porter, FD | 1 |
| Oswald, MJ | 2 |
| Palmer, DN | 2 |
| Kay, GW | 2 |
| Barwell, KJ | 1 |
| Cooper, JD | 3 |
| Bible, E | 1 |
| Gupta, P | 1 |
| Hofmann, SL | 1 |
| Sitter, B | 1 |
| Autti, T | 1 |
| Tyynelä, J | 2 |
| Sonnewald, U | 1 |
| Bathen, TF | 1 |
| Puranen, J | 1 |
| Santavuori, P | 1 |
| Haltia, MJ | 1 |
| Paetau, A | 1 |
| Polvikoski, T | 1 |
| Gribbestad, IS | 1 |
| Häkkinen, AM | 1 |
| Kopra, O | 2 |
| Vesa, J | 2 |
| von Schantz, C | 2 |
| Manninen, T | 2 |
| Minye, H | 2 |
| Fabritius, AL | 2 |
| Rapola, J | 2 |
| van Diggelen, OP | 1 |
| Saarela, J | 1 |
| Jalanko, A | 2 |
| Peltonen, L | 2 |
| Salonen, T | 1 |
| Gentile, M | 1 |
| Pears, MR | 1 |
| Mitchison, HM | 1 |
| Mortishire-Smith, RJ | 1 |
| Pearce, DA | 1 |
| Griffin, JL | 1 |
| Aberg, L | 1 |
| Talling, M | 1 |
| Härkönen, T | 1 |
| Lönnqvist, T | 1 |
| Knip, M | 1 |
| Alen, R | 1 |
| Rantala, H | 1 |
| March, PA | 2 |
| Wurzelmann, S | 2 |
| Walkley, SU | 2 |
| Schroeder, CE | 1 |
| Jolly, RD | 1 |
1 review available for gamma-aminobutyric acid and Neuronal Ceroid-Lipofuscinoses
| Article | Year |
|---|---|
Pathogenesis of brain dysfunction in Batten disease.
Topics: Animals; Atrophy; Brain; Cerebral Cortex; Dogs; gamma-Aminobutyric Acid; Golgi Apparatus; Humans; Hy | 1995 |
1 trial available for gamma-aminobutyric acid and Neuronal Ceroid-Lipofuscinoses
| Article | Year |
|---|---|
Intermittent prednisolone and autoantibodies to GAD65 in juvenile neuronal ceroid lipofuscinosis.
Topics: Adolescent; Age Factors; Autoantibodies; Biomarkers; Brain; Child; Dose-Response Relationship, Drug; | 2008 |
9 other studies available for gamma-aminobutyric acid and Neuronal Ceroid-Lipofuscinoses
| Article | Year |
|---|---|
Brain proton MR spectroscopy measurements in CLN3 disease.
Topics: Adolescent; Adult; Brain; Child; gamma-Aminobutyric Acid; Glutamic Acid; Glutamine; Humans; Magnetic | 2023 |
Location and connectivity determine GABAergic interneuron survival in the brains of South Hampshire sheep with CLN6 neuronal ceroid lipofuscinosis.
Topics: Animals; Brain; Cell Survival; gamma-Aminobutyric Acid; Interneurons; Membrane Proteins; Neural Path | 2008 |
Regional and cellular neuropathology in the palmitoyl protein thioesterase-1 null mutant mouse model of infantile neuronal ceroid lipofuscinosis.
Topics: Animals; Astrocytes; Atrophy; Disease Models, Animal; Fluorescence; gamma-Aminobutyric Acid; Hippoca | 2004 |
High-resolution magic angle spinning and 1H magnetic resonance spectroscopy reveal significantly altered neuronal metabolite profiles in CLN1 but not in CLN3.
Topics: Adolescent; Adult; Analysis of Variance; Aspartic Acid; Brain; Brain Chemistry; Child; Child, Presch | 2004 |
A mouse model for Finnish variant late infantile neuronal ceroid lipofuscinosis, CLN5, reveals neuropathology associated with early aging.
Topics: Aging; Animals; Base Sequence; Brain; Disease Models, Animal; DNA Primers; gamma-Aminobutyric Acid; | 2004 |
Mice with Ppt1Deltaex4 mutation replicate the INCL phenotype and show an inflammation-associated loss of interneurons.
Topics: Animals; Animals, Newborn; Blindness, Cortical; Cell Death; Cerebral Cortex; Disease Models, Animal; | 2005 |
High resolution 1H NMR-based metabolomics indicates a neurotransmitter cycling deficit in cerebral tissue from a mouse model of Batten disease.
Topics: Aging; Animals; Aspartic Acid; Brain; Cerebellum; Cerebral Cortex; Creatine; Disease Models, Animal; | 2005 |
Morphological alterations in neocortical and cerebellar GABAergic neurons in a canine model of juvenile Batten disease.
Topics: Animals; Cerebellum; Cerebral Cortex; Dog Diseases; Dogs; Electron Transport Complex IV; gamma-Amino | 1995 |
Changes in GABAergic neuron distribution in situ and in neuron cultures in ovine (OCL6) Batten disease.
Topics: Animals; Cells, Cultured; gamma-Aminobutyric Acid; Immunohistochemistry; Interneurons; Neocortex; Ne | 2001 |