carnitine has been researched along with BCKD Deficiency in 11 studies
| Excerpt | Relevance | Reference |
|---|---|---|
| "Maple syrup urine disease (MSUD) is an inherited aminoacidopathy caused by a deficiency in branched-chain α-keto acid dehydrogenase complex activity that leads to the accumulation of the branched-chain amino acids (BCAAs) leucine (Leu), isoleucine, and valine and their respective α-keto-acids, α-ketoisocaproic acid (KIC), α keto-β-methylvaleric acid, and α-ketoisovaleric acid." | 7.80 | Prevention of DNA damage by L-carnitine induced by metabolites accumulated in maple syrup urine disease in human peripheral leukocytes in vitro. ( Dutra-Filho, CS; Guerreiro, G; Manfredini, V; Mescka, CP; Vargas, CR; Wayhs, CA, 2014) |
| "We report on 2 women with organic acidemias, one with classical maple syrup urine disease and another with mild propionic acidemia in which protein restricted diets and carnitine supplementation were successfully employed to manage pregnancies." | 7.68 | Case reports of successful pregnancy in women with maple syrup urine disease and propionic acidemia. ( Barness, LA; Davidson, SR; Harding, CO; Van Calcar, SC; Wolff, JA, 1992) |
| "l-Carnitine (l-Car) plays a central role in the cellular energy metabolism because it transports long-chain fatty acids for oxidation and ATP generation." | 5.39 | Protein and lipid damage in maple syrup urine disease patients: l-carnitine effect. ( Biancini, GB; Dutra-Filho, CS; Guerreiro, G; Manfredini, V; Mescka, CP; Souza, C; Vanzin, CS; Vargas, CR; Wajner, M; Wayhs, CA, 2013) |
| "Maple syrup urine disease (MSUD) is an inherited aminoacidopathy caused by a deficiency in branched-chain α-keto acid dehydrogenase complex activity that leads to the accumulation of the branched-chain amino acids (BCAAs) leucine (Leu), isoleucine, and valine and their respective α-keto-acids, α-ketoisocaproic acid (KIC), α keto-β-methylvaleric acid, and α-ketoisovaleric acid." | 3.80 | Prevention of DNA damage by L-carnitine induced by metabolites accumulated in maple syrup urine disease in human peripheral leukocytes in vitro. ( Dutra-Filho, CS; Guerreiro, G; Manfredini, V; Mescka, CP; Vargas, CR; Wayhs, CA, 2014) |
| "We report on 2 women with organic acidemias, one with classical maple syrup urine disease and another with mild propionic acidemia in which protein restricted diets and carnitine supplementation were successfully employed to manage pregnancies." | 3.68 | Case reports of successful pregnancy in women with maple syrup urine disease and propionic acidemia. ( Barness, LA; Davidson, SR; Harding, CO; Van Calcar, SC; Wolff, JA, 1992) |
| "l-Carnitine (l-Car) plays a central role in the cellular energy metabolism because it transports long-chain fatty acids for oxidation and ATP generation." | 1.39 | Protein and lipid damage in maple syrup urine disease patients: l-carnitine effect. ( Biancini, GB; Dutra-Filho, CS; Guerreiro, G; Manfredini, V; Mescka, CP; Souza, C; Vanzin, CS; Vargas, CR; Wajner, M; Wayhs, CA, 2013) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 2 (18.18) | 18.2507 |
| 2000's | 0 (0.00) | 29.6817 |
| 2010's | 9 (81.82) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Bower, A | 1 |
| Imbard, A | 1 |
| Benoist, JF | 1 |
| Pichard, S | 1 |
| Rigal, O | 1 |
| Baud, O | 1 |
| Schiff, M | 1 |
| Hauschild, TC | 1 |
| Guerreiro, G | 6 |
| Mescka, CP | 6 |
| Coelho, DM | 1 |
| Steffens, L | 1 |
| Moura, DJ | 1 |
| Manfredini, V | 3 |
| Vargas, CR | 6 |
| Sharawat, IK | 1 |
| Kochar, GS | 1 |
| Saini, AG | 1 |
| Sankhyan, N | 1 |
| Wayhs, CA | 4 |
| Dutra-Filho, CS | 6 |
| Hammerschmidt, T | 1 |
| Faverzani, J | 1 |
| de Moura Coelho, D | 1 |
| Mandredini, V | 1 |
| Wajner, M | 3 |
| Donida, B | 1 |
| Marchetti, D | 1 |
| Ribas, GS | 1 |
| Coitinho, AS | 1 |
| Rosa, AP | 1 |
| Schirmbeck, G | 1 |
| da Rosa, TH | 1 |
| Catarino, F | 1 |
| de Souza, LO | 1 |
| Sitta, A | 1 |
| Mescka, C | 1 |
| Moraes, T | 1 |
| Rosa, A | 1 |
| Mazzola, P | 1 |
| Piccoli, B | 1 |
| Jacques, C | 1 |
| Dalazen, G | 1 |
| Coelho, J | 1 |
| Cortes, M | 1 |
| Terra, M | 1 |
| Regla Vargas, C | 1 |
| Vanzin, CS | 1 |
| Biancini, GB | 1 |
| Souza, C | 1 |
| Johnson, AW | 1 |
| Mills, K | 1 |
| Clayton, PT | 1 |
| Van Calcar, SC | 1 |
| Harding, CO | 1 |
| Davidson, SR | 1 |
| Barness, LA | 1 |
| Wolff, JA | 1 |
1 trial available for carnitine and BCKD Deficiency
| Article | Year |
|---|---|
DNA damage induced by alloisoleucine and other metabolites in maple syrup urine disease and protective effect of l-carnitine.
Topics: 8-Hydroxy-2'-Deoxyguanosine; Amino Acids; Carnitine; Child; Child, Preschool; Comet Assay; Deoxyguan | 2019 |
10 other studies available for carnitine and BCKD Deficiency
| Article | Year |
|---|---|
Diagnostic contribution of metabolic workup for neonatal inherited metabolic disorders in the absence of expanded newborn screening.
Topics: Amino Acids; Carnitine; Female; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intensiv | 2019 |
"Face of the Giant Panda" Sign and Temporal Cystic Changes in Maple Syrup Urine Disease.
Topics: Carnitine; Diagnosis, Differential; Diet Therapy; Female; Humans; Infant; Magnetic Resonance Imaging | 2019 |
Prevention of DNA damage by L-carnitine induced by metabolites accumulated in maple syrup urine disease in human peripheral leukocytes in vitro.
Topics: Carnitine; Comet Assay; DNA Damage; Energy Metabolism; Humans; Keto Acids; Leucine; Leukocytes; Mapl | 2014 |
L-Carnitine supplementation decreases DNA damage in treated MSUD patients.
Topics: Carnitine; Child; Child, Preschool; DNA Damage; Female; Humans; Leukocytes; Male; Maple Syrup Urine | 2015 |
Investigation of inflammatory profile in MSUD patients: benefit of L-carnitine supplementation.
Topics: Carnitine; Child; Child, Preschool; Dietary Supplements; Female; Humans; Inflammation; Inflammation | 2015 |
L-carnitine Prevents Oxidative Stress in the Brains of Rats Subjected to a Chemically Induced Chronic Model of MSUD.
Topics: Amino Acids, Branched-Chain; Animals; Brain; Carnitine; Catalase; Disease Models, Animal; Glutathion | 2016 |
In vivo neuroprotective effect of L-carnitine against oxidative stress in maple syrup urine disease.
Topics: 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide); Animals; Antioxidants; Carnitine; Catalase; Cereb | 2011 |
Protein and lipid damage in maple syrup urine disease patients: l-carnitine effect.
Topics: Amino Acids; Analysis of Variance; Carnitine; Child; Child, Preschool; Female; Humans; Lipid Metabol | 2013 |
The use of automated electrospray ionization tandem MS for the diagnosis of inborn errors of metabolism from dried blood spots.
Topics: Biliary Atresia; Carnitine; Child; Fatty Acid Desaturases; Humans; Infant, Newborn; Maple Syrup Urin | 1996 |
Case reports of successful pregnancy in women with maple syrup urine disease and propionic acidemia.
Topics: Adult; Amino Acids; Carbon-Carbon Ligases; Carnitine; Citrates; Citric Acid; Female; Fetal Growth Re | 1992 |