carnitine has been researched along with Neuronal Ceroid-Lipofuscinoses in 9 studies
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 |
|---|---|---|
| "These data on disease progression will be useful in future studies using the canine model for therapeutic intervention studies." | 1.72 | Characterization of neurological disease progression in a canine model of CLN5 neuronal ceroid lipofuscinosis. ( Coates, JR; Jensen, CA; Katz, ML; Kick, GR; Meiman, EJ, 2022) |
| "Carnitine supplement was administrated in two patients, which resulted in resolution of symptoms and normalized ammonium levels." | 1.40 | Valproate-induced hyperammonemia in juvenile ceroid lipofuscinosis (Batten disease). ( Larsen, EP; Ostergaard, JR, 2014) |
| "L-carnitine treatment increased the mitochondrial enzyme activities in cortical regions towards control value and was effective in enhancing QO2 and decreasing TBARS levels in the spinal cord of mndT." | 1.31 | Mitochondrial oxidative metabolism in motor neuron degeneration (mnd) mouse central nervous system. ( Bertamini, M; Bigini, P; Curti, D; Guarneri, P; Guarneri, R; Marzani, B; Mennini, T, 2002) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 3 (33.33) | 18.2507 |
| 2000's | 4 (44.44) | 29.6817 |
| 2010's | 1 (11.11) | 24.3611 |
| 2020's | 1 (11.11) | 2.80 |
| Authors | Studies |
|---|---|
| Meiman, EJ | 1 |
| Kick, GR | 1 |
| Jensen, CA | 1 |
| Coates, JR | 1 |
| Katz, ML | 6 |
| Larsen, EP | 1 |
| Ostergaard, JR | 1 |
| Bertamini, M | 1 |
| Marzani, B | 1 |
| Guarneri, R | 1 |
| Guarneri, P | 1 |
| Bigini, P | 1 |
| Mennini, T | 1 |
| Curti, D | 1 |
| Siakotos, AN | 2 |
| Rice, LM | 1 |
| Gao, CL | 1 |
| Infante, JP | 1 |
| Huszagh, VA | 1 |
| Hutchins, GD | 1 |
| Farlow, MR | 1 |
| Sanders, DA | 1 |
| Sanders, DN | 1 |
| Hansen, EA | 1 |
| Johnson, GS | 1 |
1 review available for carnitine and Neuronal Ceroid-Lipofuscinoses
| Article | Year |
|---|---|
Secondary carnitine deficiency and impaired docosahexaenoic (22:6n-3) acid synthesis: a common denominator in the pathophysiology of diseases of oxidative phosphorylation and beta-oxidation.
Topics: Animals; Carnitine; Disease Models, Animal; Docosahexaenoic Acids; Humans; Metabolism, Inborn Errors | 2000 |
8 other studies available for carnitine and Neuronal Ceroid-Lipofuscinoses
| Article | Year |
|---|---|
Characterization of neurological disease progression in a canine model of CLN5 neuronal ceroid lipofuscinosis.
Topics: Animals; Atrophy; Carnitine; Disease Progression; Dogs; Homozygote; Nervous System Diseases; Neurona | 2022 |
Valproate-induced hyperammonemia in juvenile ceroid lipofuscinosis (Batten disease).
Topics: Adolescent; Anticonvulsants; Brain Diseases; Carnitine; Drug Therapy, Combination; Female; Humans; H | 2014 |
Mitochondrial oxidative metabolism in motor neuron degeneration (mnd) mouse central nervous system.
Topics: Age Factors; Amyotrophic Lateral Sclerosis; Animals; Carnitine; Central Nervous System; Cerebral Cor | 2002 |
Canine hereditary ceroid-lipofuscinosis: evidence for a defect in the carnitine biosynthetic pathway.
Topics: Animals; Biomarkers; Carnitine; Dog Diseases; Dogs; Genetic Carrier Screening; Homozygote; Humans; L | 1995 |
Decreased plasma carnitine and trimethyl-L-lysine levels associated with lysosomal accumulation of a trimethyl-L-lysine containing protein in Batten disease.
Topics: Adult; Carnitine; Child; Female; Heterozygote; Humans; Lysine; Male; Neuronal Ceroid-Lipofuscinoses | 1996 |
Dietary carnitine supplements slow disease progression in a putative mouse model for hereditary ceroid-lipofuscinosis.
Topics: Animals; Brain Chemistry; Carnitine; Cerebral Cortex; Disease Models, Animal; Lysosomes; Male; Mice; | 1997 |
Assessment of dietary therapies in a canine model of Batten disease.
Topics: Animals; Carnitine; Cerebral Ventricles; Corn Oil; Disease Models, Animal; Dogs; Fish Oils; Glucose; | 2001 |
Assessment of plasma carnitine concentrations in relation to ceroid lipofuscinosis in Tibetan Terriers.
Topics: Age Factors; Animals; Carnitine; Cerebral Cortex; Dog Diseases; Dogs; Female; Male; Microscopy, Elec | 2002 |