carnitine has been researched along with Cardiac Hypertrophy in 45 studies
Cardiac Hypertrophy: Enlargement of the HEART due to chamber HYPERTROPHY, an increase in wall thickness without an increase in the number of cells (MYOCYTES, CARDIAC). It is the result of increase in myocyte size, mitochondrial and myofibrillar mass, as well as changes in extracellular matrix.
| Excerpt | Relevance | Reference |
|---|---|---|
| " Secondary carnitine deficiency, which is frequently observed in hemodialysis patients, has been associated with cardiac hypertrophy and heart failure and may impair myocardial fatty acid oxidation." | 7.74 | Myocardial function, energy provision, and carnitine deficiency in experimental uremia. ( Bhandari, S; Reddy, V; Seymour, AM, 2007) |
| "The juvenile visceral steatosis (JVS) mouse exhibits hereditary systemic carnitine deficiency and develops cardiac hypertrophy." | 7.70 | Characteristics of cardiac hypertrophy in the juvenile visceral steatosis mouse with systemic carnitine deficiency. ( Hanafusa, T; Hayakawa, J; Hayashi, M; Hotta, K; Ishiguro, K; Kuwajima, M; Lu, K; Matsuzawa, Y; Miyagawa, J; Murakami, T; Nakajima, H; Namba, M; Narama, I; Nikaido, H; Okita, K; Ono, A; Ozaki, K; Sei, M; Shima, K, 1998) |
| "The long-term administration of L-carnitine was very effective in preventing cardiomegaly in juvenile visceral steatosis (JVS) mice, which was confirmed by heart weight as well as the lipid contents in heart tissue." | 7.70 | Cardiomegaly in the juvenile visceral steatosis (JVS) mouse is reduced with acute elevation of heart short-chain acyl-carnitine level after L-carnitine injection. ( Harashima, H; Hayashi, M; Horiuchi, M; Kamido, H; Kudo, T; Kuwajima, M; Lu, K; Ono, A; Ozaki, K; Saheki, T; Sei, M; Shima, K, 1999) |
| "To clarify the mechanism of cardiac hypertrophy in carnitine-deficient JVS mice, we studied the possible role of catecholamine metabolism." | 7.70 | Catecholamine metabolism inhibitors and receptor blockades only partially suppress cardiac hypertrophy of juvenile visceral steatosis mice with systemic carnitine deficiency. ( Horiuchi, M; Jalil, A; Kobayashi, K; Nomoto, M; Saheki, T, 1999) |
| "Carnitine deficiency in the serum was found in 5 infants with cystic fibrosis, impaired liver function and neurological symptoms." | 7.69 | [Total carnitine level in infants with cystic fibrosis and deficit supplementation by means of pharmacologic preparations and diet. Introductory remarks]. ( Bujniewicz, E; Chlebowczyk, U; Krauze, M; Maliszewska, I; Mastalerz, Z; Szymańska, M; Woś, H, 1995) |
| "The discovery of cardiac hypertrophy in carnitine-deficient jvs mice will lead to clarification of the pathophysiology of cardiomyopathy in systemic carnitine deficiency in human beings." | 5.29 | Cardiac hypertrophy in juvenile visceral steatosis (jvs) mice with systemic carnitine deficiency. ( Hayakawa, J; Horiuchi, M; Kobayashi, K; Koizumi, T; Kuriwaki, K; Kuwajima, M; Nikaido, H; Tomomura, M; Yoshida, H; Yoshimine, K, 1993) |
| "Cardiac hypertrophy was induced by abdominal aorta constriction in rats." | 5.28 | Hemodynamic and metabolic activities of propionyl-L-carnitine in rats with pressure-overload cardiac hypertrophy. ( Benatti, P; Bianchi, G; Cardace, G; English, E; Micheletti, R; Motterlini, R; Samaja, M; Tarantola, M; Yang, XP, 1992) |
| " Primary outcome measures were mitochondrial ATP production measured as phosphocreatine recovery by 31Phosphorus magnetic resonance spectroscopy, neurological deficits assessed by the international co-operative ataxia rating scale and cardiac hypertrophy in echocardiography." | 5.11 | L-carnitine and creatine in Friedreich's ataxia. A randomized, placebo-controlled crossover trial. ( Abele, M; Colier, WN; Klockgether, T; Kuntz-Hehner, S; Müller, K; Przuntek, H; Schillings, M; Schöls, L; Skipka, G; van Beekvelt, MC; Vorgerd, M; Zange, J, 2005) |
| " Secondary carnitine deficiency, which is frequently observed in hemodialysis patients, has been associated with cardiac hypertrophy and heart failure and may impair myocardial fatty acid oxidation." | 3.74 | Myocardial function, energy provision, and carnitine deficiency in experimental uremia. ( Bhandari, S; Reddy, V; Seymour, AM, 2007) |
| " At 4 weeks after ascending aortic constriction, jvs/+ mice showed an exaggeration of cardiac hypertrophy and pulmonary congestion, further increased gene expression of atrial natriuretic peptide in the left ventricles, further deterioration of left ventricular fractional shortening, reduced myocardial phosphocreatine:adenosine triphosphate ratio, and increased mortality compared with wild-type mice; l-carnitine supplementation prevented these changes in jvs/+ mice subjected to ascending aortic constriction." | 3.74 | Pressure overload-induced cardiomyopathy in heterozygous carrier mice of carnitine transporter gene mutation. ( Asai, T; Matsui, H; Murakami, H; Murakami, R; Murohara, T; Numaguchi, Y; Okumura, K; Takahashi, R; Tsuzuki, M, 2007) |
| "The juvenile visceral steatosis (JVS) mouse, a genetic model of systemic carnitine deficiency resulting from carnitine transport mutation, develops cardiac hypertrophy." | 3.72 | Changes in distinct species of 1,2-diacylglycerol in cardiac hypertrophy due to energy metabolic disorder. ( Hayashi, K; Ito, M; Kamiya, H; Matsubara, K; Matsui, H; Okumura, K; Saburi, Y; Takahashi, R, 2003) |
| "To clarify the pathogenesis of cardiac hypertrophy in carnitine-deficient juvenile visceral steatosis (JVS) mice, we performed differential mRNA display analysis with the ventricles of control and JVS mice." | 3.71 | Novel mRNA molecules are induced in hypertrophied ventricles of carnitine-deficient mice and belong to a family of up-regulated gene in cells overexpressing c-erbB-2. ( Begum, L; Fukumaru, S; Higashi, M; Horiuchi, M; Iijima, M; Jalil, MA; Kanzaki, T; Kobayashi, K; Masuda, M; Saheki, T; Wakana, S, 2002) |
| "The juvenile visceral steatosis (JVS) mouse exhibits hereditary systemic carnitine deficiency and develops cardiac hypertrophy." | 3.70 | Characteristics of cardiac hypertrophy in the juvenile visceral steatosis mouse with systemic carnitine deficiency. ( Hanafusa, T; Hayakawa, J; Hayashi, M; Hotta, K; Ishiguro, K; Kuwajima, M; Lu, K; Matsuzawa, Y; Miyagawa, J; Murakami, T; Nakajima, H; Namba, M; Narama, I; Nikaido, H; Okita, K; Ono, A; Ozaki, K; Sei, M; Shima, K, 1998) |
| "The long-term administration of L-carnitine was very effective in preventing cardiomegaly in juvenile visceral steatosis (JVS) mice, which was confirmed by heart weight as well as the lipid contents in heart tissue." | 3.70 | Cardiomegaly in the juvenile visceral steatosis (JVS) mouse is reduced with acute elevation of heart short-chain acyl-carnitine level after L-carnitine injection. ( Harashima, H; Hayashi, M; Horiuchi, M; Kamido, H; Kudo, T; Kuwajima, M; Lu, K; Ono, A; Ozaki, K; Saheki, T; Sei, M; Shima, K, 1999) |
| "To clarify the mechanism of cardiac hypertrophy in carnitine-deficient JVS mice, we studied the possible role of catecholamine metabolism." | 3.70 | Catecholamine metabolism inhibitors and receptor blockades only partially suppress cardiac hypertrophy of juvenile visceral steatosis mice with systemic carnitine deficiency. ( Horiuchi, M; Jalil, A; Kobayashi, K; Nomoto, M; Saheki, T, 1999) |
| " Significant laboratory findings included elevated plasma levels of creatine phosphokinase and acyl-carnitine and a fatty liver at biopsy suggesting a diagnosis of VLCAD deficiency." | 3.70 | Milder childhood form of very long-chain acyl-CoA dehydrogenase deficiency in a 6-year-old Japanese boy. ( Abo, W; Doi, T; Fukao, T; Hayashi, K; Hori, T; Nakada, T; Takahashi, Y; Tateno, M; Terada, N, 2000) |
| "Alterations in energy metabolism, reduced fatty acid oxidation, and cardiac carnitine content have been implicated in the evolution from compensated to decompensated cardiac hypertrophy." | 3.69 | Alterations in energy metabolism of hypertrophied rat cardiomyocytes: influence of propionyl-L-carnitine. ( Anversa, P; Bianchi, G; Ceppi, E; Cinato, E; Conti, F; Ferrari, P; Torielli, L, 1995) |
| "Carnitine deficiency in the serum was found in 5 infants with cystic fibrosis, impaired liver function and neurological symptoms." | 3.69 | [Total carnitine level in infants with cystic fibrosis and deficit supplementation by means of pharmacologic preparations and diet. Introductory remarks]. ( Bujniewicz, E; Chlebowczyk, U; Krauze, M; Maliszewska, I; Mastalerz, Z; Szymańska, M; Woś, H, 1995) |
| "To characterize cardiac hypertrophy in juvenile visceral steatosis (JVS) mice with systemic carnitine deficiency, we investigated how the hypertrophy develops and whether it is associated with altered expression of any specific genes, especially atrial natriuretic peptide (ANP) and contractile protein genes, in the hypertrophied ventricle." | 3.69 | Altered expression of atrial natriuretic peptide and contractile protein genes in hypertrophied ventricle of JVS mice with systemic carnitine deficiency. ( Abdul, JM; Horiuchi, M; Itoh, H; Kobayashi, K; Masuda, M; Nakao, K; Ogawa, Y; Osame, M; Saheki, T; Suzuki, S; Tomomura, M; Yoshimine, K, 1997) |
| "In order to clarify the pathogenesis and pathophysiology of cardiac hypertrophy in carnitine-deficient juvenile visceral steatosis (JVS) mice, we performed mRNA differential display analysis with total RNA extracted from the ventricles of control and JVS mice at 14 days of age." | 3.69 | A novel gene suppressed in the ventricle of carnitine-deficient juvenile visceral steatosis mice. ( Horiuchi, M; Kobayashi, K; Masuda, M; Saheki, T; Terazono, H; Yoshimura, N, 1997) |
| "Aim of this study was to assess the effect of propionyl-L-carnitine (PLC), a naturally occurring derivative of L-carnitine, in cardiac hypertrophy induced by pressure overload in rats." | 3.68 | Functional and metabolic effects of propionyl-L-carnitine in the isolated perfused hypertrophied rat heart. ( Bianchi, G; Micheletti, R; Motterlini, R; Samaja, M; Tarantola, M, 1992) |
| "Cardiac hypertrophy was induced in rabbits by subcutaneous injection of thyroxine or isoprenaline or by surgically constricting the abdominal aorta." | 3.66 | Metabolism of lipids in experimental hypertrophic hearts of rabbits. ( Cameron, AJ; Revis, NW, 1979) |
| "glycogenosis type II and III), lysosomal storage diseases (e." | 2.41 | Metabolic cardiomyopathies. ( Guertl, B; Hoefler, G; Noehammer, C, 2000) |
| "Antioxidant changes in juvenile visceral steatosis (JVS) mice, a model of heart hypertrophy due to disorder of fatty-acid oxidation, were examined at 4 weeks (developing hypertrophy stage) and 8 weeks of age (established hypertrophy stage)." | 1.31 | Antioxidant changes in the hypertrophied heart due to energy metabolic disorder. ( Hayakawa, T; Hayashi, K; Ito, M; Kamiya, H; Matsui, H; Okumura, K; Saburi, Y, 2001) |
| " Long-term administration of propionyl-L-carnitine normalized the degree of reduction of mitochondrial pyridine nucleotides and improved the kinetics of mitochondrial ATP production in volume-overloaded hearts." | 1.30 | Control of oxidative metabolism in volume-overloaded rat hearts: effect of propionyl-L-carnitine. ( El Alaoui-Talibi, Z; Guendouz, A; Moravec, J; Moravec, M, 1997) |
| "The discovery of cardiac hypertrophy in carnitine-deficient jvs mice will lead to clarification of the pathophysiology of cardiomyopathy in systemic carnitine deficiency in human beings." | 1.29 | Cardiac hypertrophy in juvenile visceral steatosis (jvs) mice with systemic carnitine deficiency. ( Hayakawa, J; Horiuchi, M; Kobayashi, K; Koizumi, T; Kuriwaki, K; Kuwajima, M; Nikaido, H; Tomomura, M; Yoshida, H; Yoshimine, K, 1993) |
| "Carnitine content was significantly decreased in hypertrophied hearts compared to control hearts, but was normalized by propionyl L-carnitine treatment." | 1.29 | Propionyl L-carnitine improvement of hypertrophied rat heart function is associated with an increase in cardiac efficiency. ( Allard, MF; Lopaschuk, GD; Schönekess, BO, 1995) |
| "Cardiac hypertrophy was induced by abdominal aorta constriction in rats." | 1.28 | Hemodynamic and metabolic activities of propionyl-L-carnitine in rats with pressure-overload cardiac hypertrophy. ( Benatti, P; Bianchi, G; Cardace, G; English, E; Micheletti, R; Motterlini, R; Samaja, M; Tarantola, M; Yang, XP, 1992) |
| "Carnitine metabolism was examined in spontaneously hypertensive rats (SHR)." | 1.27 | Altered carnitine metabolism in spontaneously hypertensive rats. ( Foster, KA; O'Rourke, B; Reibel, DK, 1985) |
| "Treatment with prednisone resulted in clinical improvement but no change in muscle histology." | 1.25 | Hereditary carnitine deficiency of muscle. ( Dimauro, S; Griggs, RC; Markesbery, W; VanDyke, DH, 1975) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 12 (26.67) | 18.7374 |
| 1990's | 19 (42.22) | 18.2507 |
| 2000's | 10 (22.22) | 29.6817 |
| 2010's | 2 (4.44) | 24.3611 |
| 2020's | 2 (4.44) | 2.80 |
| Authors | Studies |
|---|---|
| Vaughan, OR | 1 |
| Rosario, FJ | 1 |
| Chan, J | 1 |
| Cox, LA | 1 |
| Ferchaud-Roucher, V | 1 |
| Zemski-Berry, KA | 1 |
| Reusch, JEB | 1 |
| Keller, AC | 1 |
| Powell, TL | 1 |
| Jansson, T | 1 |
| Tan, YY | 1 |
| Fong, WYN | 1 |
| Chan, CJ | 1 |
| Chandran, S | 1 |
| Horiuchi, M | 8 |
| Nakakuma, M | 1 |
| Arimura, E | 1 |
| Ushikai, M | 1 |
| Yoshida, G | 1 |
| Kolwicz, SC | 1 |
| Olson, DP | 1 |
| Marney, LC | 1 |
| Garcia-Menendez, L | 1 |
| Synovec, RE | 1 |
| Tian, R | 1 |
| Fukumaru, S | 1 |
| Kobayashi, K | 6 |
| Jalil, MA | 1 |
| Iijima, M | 1 |
| Masuda, M | 4 |
| Begum, L | 1 |
| Higashi, M | 1 |
| Wakana, S | 1 |
| Kanzaki, T | 1 |
| Saheki, T | 6 |
| Buziashvili, IuI | 1 |
| Kliuchnikov, IV | 1 |
| Melkonian, AM | 1 |
| Inozemtseva, EV | 1 |
| Kovalenko, OA | 1 |
| Mamaev, KhK | 1 |
| Saburi, Y | 2 |
| Okumura, K | 3 |
| Matsui, H | 3 |
| Hayashi, K | 3 |
| Kamiya, H | 2 |
| Takahashi, R | 2 |
| Matsubara, K | 1 |
| Ito, M | 2 |
| Suenaga, M | 1 |
| Kuwajima, M | 4 |
| Himeda, T | 1 |
| Morokami, K | 1 |
| Matsuura, T | 1 |
| Ozaki, K | 3 |
| Arakaki, N | 1 |
| Shibata, H | 1 |
| Higuti, T | 1 |
| Schöls, L | 1 |
| Zange, J | 1 |
| Abele, M | 1 |
| Schillings, M | 1 |
| Skipka, G | 1 |
| Kuntz-Hehner, S | 1 |
| van Beekvelt, MC | 1 |
| Colier, WN | 1 |
| Müller, K | 1 |
| Klockgether, T | 1 |
| Przuntek, H | 1 |
| Vorgerd, M | 1 |
| Reddy, V | 1 |
| Bhandari, S | 1 |
| Seymour, AM | 1 |
| Asai, T | 1 |
| Murakami, H | 1 |
| Murakami, R | 1 |
| Tsuzuki, M | 1 |
| Numaguchi, Y | 1 |
| Murohara, T | 1 |
| Drüeke, T | 1 |
| Reibel, DK | 3 |
| Uboh, CE | 1 |
| Kent, RL | 1 |
| Kudoh, Y | 1 |
| Shoji, T | 1 |
| Oimatsu, H | 1 |
| Yoshida, S | 1 |
| Kikuchi, K | 1 |
| Iimura, O | 1 |
| Bowe, C | 1 |
| Nzonzi, J | 1 |
| Corsin, A | 1 |
| Moravec, J | 3 |
| Feuvray, D | 1 |
| Schönekess, BO | 2 |
| Allard, MF | 2 |
| Lopaschuk, GD | 2 |
| Reggiani, C | 2 |
| Canepari, M | 2 |
| Micheletti, R | 5 |
| Cappelli, V | 1 |
| Zanardi, MC | 1 |
| Hülsmann, WC | 1 |
| Peschechera, A | 1 |
| Schneijdenberg, CT | 1 |
| Verkleij, AJ | 1 |
| Giacalone, G | 2 |
| Salardi, S | 1 |
| Bianchi, G | 5 |
| Yoshida, H | 1 |
| Kuriwaki, K | 1 |
| Yoshimine, K | 2 |
| Tomomura, M | 2 |
| Koizumi, T | 1 |
| Nikaido, H | 2 |
| Hayakawa, J | 2 |
| el Alaoui-Talibi, Z | 2 |
| Bouhaddioni, N | 1 |
| Torielli, L | 1 |
| Conti, F | 1 |
| Cinato, E | 1 |
| Ceppi, E | 1 |
| Anversa, P | 1 |
| Ferrari, P | 1 |
| Woś, H | 1 |
| Krauze, M | 1 |
| Bujniewicz, E | 1 |
| Chlebowczyk, U | 1 |
| Mastalerz, Z | 1 |
| Szymańska, M | 1 |
| Maliszewska, I | 1 |
| Guendouz, A | 1 |
| Moravec, M | 1 |
| Suzuki, S | 1 |
| Abdul, JM | 1 |
| Ogawa, Y | 1 |
| Itoh, H | 1 |
| Nakao, K | 1 |
| Osame, M | 1 |
| Terazono, H | 2 |
| Yoshimura, N | 1 |
| Lu, K | 2 |
| Sei, M | 2 |
| Ono, A | 2 |
| Hayashi, M | 2 |
| Ishiguro, K | 1 |
| Hotta, K | 1 |
| Okita, K | 1 |
| Murakami, T | 1 |
| Miyagawa, J | 1 |
| Narama, I | 1 |
| Nakajima, H | 1 |
| Namba, M | 1 |
| Hanafusa, T | 1 |
| Matsuzawa, Y | 1 |
| Shima, K | 2 |
| Harashima, H | 1 |
| Kudo, T | 1 |
| Kamido, H | 1 |
| Jalil, A | 1 |
| Nomoto, M | 1 |
| Doi, T | 1 |
| Abo, W | 1 |
| Tateno, M | 1 |
| Hori, T | 1 |
| Nakada, T | 1 |
| Fukao, T | 1 |
| Takahashi, Y | 1 |
| Terada, N | 1 |
| Guertl, B | 1 |
| Noehammer, C | 1 |
| Hoefler, G | 1 |
| Hayakawa, T | 1 |
| VanDyke, DH | 1 |
| Griggs, RC | 1 |
| Markesbery, W | 1 |
| Dimauro, S | 1 |
| Revis, NW | 1 |
| Cameron, AJ | 1 |
| Yang, XP | 1 |
| Samaja, M | 2 |
| English, E | 1 |
| Benatti, P | 1 |
| Tarantola, M | 2 |
| Cardace, G | 1 |
| Motterlini, R | 2 |
| Whitmer, JT | 1 |
| O'Rourke, B | 2 |
| Foster, KA | 2 |
| Lübbecke, F | 1 |
| Steudle, V | 1 |
| Schaper, J | 1 |
| Wizemann, V | 1 |
| Wittels, B | 1 |
| Spann, JF | 1 |
| Böhmer, T | 1 |
| Rydning, A | 1 |
| Solberg, HE | 1 |
| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| In Vivo Study of Safety, Tolerability and Dosing Effect on SMN mRNA and Protein Levels of Valproic Acid in Patients With Spinal Muscular Atrophy[NCT00374075] | Phase 1 | 42 participants | Interventional | 2003-09-30 | Completed | ||
| Phase I/II Trial of Valproic Acid and Carnitine in Infants With Spinal Muscular Atrophy Type I (CARNI-VAL Type I)[NCT00661453] | Phase 1/Phase 2 | 40 participants (Actual) | Interventional | 2008-04-30 | Completed | ||
| Multi-center Phase II Trial of Valproic Acid and Carnitine in Patients With Spinal Muscular Atrophy (SMA CARNI-VAL Trial)[NCT00227266] | Phase 2 | 94 participants (Actual) | Interventional | 2005-09-30 | Completed | ||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
(NCT00661453)
Timeframe: -2 weeks, time 0, 3 months, 6 months
| Intervention | g (Mean) | |||||
|---|---|---|---|---|---|---|
| Lean Mass Baseline | Lean Mass 3 months | Lean Mass 6 months | Fat Mass Baseline | Fat Mass 3 months | Fat Mass 6 months | |
| SMA Type 1 | 4317.15 | 4993.92 | 5133.83 | 3011.37 | 3618.25 | 4316.08 |
The maximum Compound Motor Action Potential (CMAP) is a measurement obtained through EMG testing that is associated with disease progression. In this study, we measure the maximum CMAP by stimulating one nerve in the hand and measuring the response of the muscle. This is done multiple times, the outcome used is the highest peak, or response observed. (NCT00227266)
Timeframe: 1 month prior to official enrollment, beginning of study (0 months), 6 months, 12 months (data point not available)
| Intervention | mV (Mean) | |
|---|---|---|
| Baseline | 6 months | |
| Cohort 1a Sitters Placebo Then Treatment | 2.28 | 2.32 |
| Cohort 1b Sitters Treatment | 2.93 | 2.37 |
| Cohort 2 Standers and Walkers - Treatment | 5.52 | 6.56 |
The maximum Compound Motor Action Potential (CMAP) is a measurement obtained through EMG testing that is associated with disease progression. In this study, we measure the maximum CMAP by stimulating one nerve in the hand and measuring the response of the muscle. This is done multiple times, the outcome used is the highest peak, or response observed. (NCT00227266)
Timeframe: 1 month prior to official enrollment, beginning of study (0 months), 6 months, 12 months (data point not available)
| Intervention | mV (Median) | |
|---|---|---|
| Baseline | 6 months | |
| Cohort 1a Sitters Placebo Then Treatment | 1.91 | 1.44 |
| Cohort 1b Sitters Treatment | 2.2 | 1.8 |
| Cohort 2 Standers and Walkers - Treatment | 5.3 | 5.85 |
The maximum Compound Motor Action Potential (CMAP) area is a measurement obtained through EMG testing that is associated with disease progression. In this study, we measure the maximum CMAP by stimulating one nerve in the hand and measuring the response of the muscle. This procedure is repeated multiple times. The maximum area is the response that results in the largest area under the response curve. (NCT00227266)
Timeframe: 1 month prior to official enrollment, beginning of study (0 months), 6 months, 12 months (data point not available)
| Intervention | mVms (Mean) | |
|---|---|---|
| Baseline | 6 months | |
| Cohort 1a Sitters Placebo Then Treatment | 5.46 | 5.28 |
| Cohort 1b Sitters Treatment | 5.45 | 5.26 |
| Cohort 2 Standers and Walkers - Treatment | 14.85 | 16.26 |
The maximum Compound Motor Action Potential (CMAP) area is a measurement obtained through EMG testing that is associated with disease progression. In this study, we measure the maximum CMAP by stimulating one nerve in the hand and measuring the response of the muscle. This procedure is repeated multiple times. The maximum area is the response that results in the largest area under the response curve. (NCT00227266)
Timeframe: 1 month prior to official enrollment, beginning of study (0 months), 6 months, 12 months (data point not available)
| Intervention | mVms (Median) | |
|---|---|---|
| Baseline | 6 months | |
| Cohort 1a Sitters Placebo Then Treatment | 3.6 | 3.74 |
| Cohort 1b Sitters Treatment | 4.6 | 3.4 |
| Cohort 2 Standers and Walkers - Treatment | 13.65 | 16.85 |
Comparison of Modified Hammersmith Change from baseline to 6 months. Scores range from 0 to 40. A higher score indicates a better outcome. This scale is used to assess gross motor abilities of non-ambulant children with SMA in multiple research trials as well as in clinical settings. (NCT00227266)
Timeframe: 0 months, 6 months
| Intervention | Score (Mean) | ||
|---|---|---|---|
| Baseline visit (0 weeks) | 6 Month visit (V2) | Change from Baseline | |
| Cohort 1a Sitters Placebo Then Treatment | 20.0 | 20.6 | 0.6 |
| Cohort 1b Sitters Treatment | 16.6 | 16.8 | 0.2 |
"Baseline Modified Hammersmith Extend testing. The baseline test is the score they receive during their screening visits. This scale ranges from 0 to 56. A higher score indicates a better outcome.~This scale is used to assess gross motor abilities of children with SMA in multiple research trials as well as in clinical settings." (NCT00227266)
Timeframe: 1 month prior to enrollment, at enrollment (0 months)
| Intervention | Score (Mean) | |
|---|---|---|
| Modified Hammersmith Extend at S1 (-4 weeks) | Modified Hammersmith Extend at S2 (0 weeks) | |
| Cohort 2 Experimental | 47.0 | 48.3 |
3 reviews available for carnitine and Cardiac Hypertrophy
| Article | Year |
|---|---|
[Experimental Approach to Analysis of the Relationship between Food Environments and Lifestyle-Related Diseases, Including Cardiac Hypertrophy, Fatty Liver, and Fatigue Symptoms].
Topics: Animals; Beriberi; Cardiomegaly; Carnitine; Disease Models, Animal; Fatigue Syndrome, Chronic; Fatty | 2015 |
[Ischemic remodeling of the left ventricle (definition, pathogenesis, diagnosis, medical and surgical correction)].
Topics: Adrenergic beta-Antagonists; Angioplasty, Balloon, Coronary; Angiotensin-Converting Enzyme Inhibitor | 2002 |
Metabolic cardiomyopathies.
Topics: Adult; Animals; Calcium; Cardiomegaly; Cardiomyopathies; Cardiomyopathy, Alcoholic; Carnitine; Diabe | 2000 |
1 trial available for carnitine and Cardiac Hypertrophy
| Article | Year |
|---|---|
L-carnitine and creatine in Friedreich's ataxia. A randomized, placebo-controlled crossover trial.
Topics: Adenosine Triphosphate; Adolescent; Adult; Cardiomegaly; Carnitine; Child; Creatine; Female; Friedre | 2005 |
41 other studies available for carnitine and Cardiac Hypertrophy
| Article | Year |
|---|---|
Maternal obesity causes fetal cardiac hypertrophy and alters adult offspring myocardial metabolism in mice.
Topics: Adult Children; Animals; Cardiomegaly; Cardiovascular Diseases; Carnitine; Female; Fetal Heart; Hear | 2022 |
Do renal and cardiac malformations in the fetus signal carnitine palmitoyltransferase II deficiency? A rare lethal fatty acid oxidation defect.
Topics: Cardiomegaly; Carnitine; Carnitine O-Palmitoyltransferase; Fatty Acids; Female; Fetus; Humans; Infan | 2022 |
Cardiac-specific deletion of acetyl CoA carboxylase 2 prevents metabolic remodeling during pressure-overload hypertrophy.
Topics: Acetyl-CoA Carboxylase; Animals; Aorta; Blotting, Western; Cardiomegaly; Carnitine; Constriction, Pa | 2012 |
Novel mRNA molecules are induced in hypertrophied ventricles of carnitine-deficient mice and belong to a family of up-regulated gene in cells overexpressing c-erbB-2.
Topics: Amino Acid Sequence; Animals; Base Sequence; Cardiomegaly; Carnitine; Cloning, Molecular; Exons; Gen | 2002 |
Changes in distinct species of 1,2-diacylglycerol in cardiac hypertrophy due to energy metabolic disorder.
Topics: Animals; Cardiomegaly; Carnitine; Ceramides; Chromatography, Thin Layer; Diglycerides; Echocardiogra | 2003 |
Identification of the up- and down-regulated genes in the heart of juvenile visceral steatosis mice.
Topics: Animals; Cardiomegaly; Carnitine; Disease Models, Animal; Down-Regulation; Gene Expression Profiling | 2004 |
Myocardial function, energy provision, and carnitine deficiency in experimental uremia.
Topics: Animals; Cardiomegaly; Cardiovascular Diseases; Carnitine; Creatinine; Disease Models, Animal; Energ | 2007 |
Pressure overload-induced cardiomyopathy in heterozygous carrier mice of carnitine transporter gene mutation.
Topics: Adenosine Triphosphate; Animals; Aorta; Atrial Natriuretic Factor; Blood Pressure; Cardiomegaly; Car | 2007 |
[Uremic cardiomyopathy].
Topics: Calcium; Calcium Phosphates; Cardiomegaly; Cardiomyopathies; Carnitine; Humans; Hyperparathyroidism; | 1981 |
Altered coenzyme A and carnitine metabolism in pressure-overload hypertrophied hearts.
Topics: Animals; Body Weight; Cardiac Output; Cardiomegaly; Carnitine; Cats; Coenzyme A; Female; Heart Ventr | 1983 |
The role of L-carnitine in the pathogenesis of cardiomegaly in patients with chronic hemodialysis.
Topics: Adolescent; Adult; Aged; Cardiomegaly; Carnitine; Cholesterol; Cholesterol, HDL; Chronic Disease; Fe | 1983 |
Lipid intermediates in chronically volume-overloaded rat hearts. Effect of diffuse ischemia.
Topics: Acyl Coenzyme A; Adenosine Triphosphate; Animals; Cardiomegaly; Carnitine; Coenzyme A; Coronary Dise | 1984 |
Propionyl L-carnitine improvement of hypertrophied heart function is accompanied by an increase in carbohydrate oxidation.
Topics: Animals; Carbohydrate Metabolism; Cardiomegaly; Cardiotonic Agents; Carnitine; Male; Oxidation-Reduc | 1995 |
Effect of propionyl-L-carnitine (PLC) on the kinetic properties of the myofibrillar system in pressure overload cardiac hypertrophy.
Topics: Animals; Cardiomegaly; Cardiotonic Agents; Carnitine; Heart; Heart Ventricles; Kinetics; Male; Myoca | 1995 |
Comparison of the effects of carnitine palmitoyltransferase-1 and -2 inhibitors on rat heart hypertrophy.
Topics: Animals; Betaine; Cardiomegaly; Carnitine; Carnitine Acyltransferases; Carnitine O-Palmitoyltransfer | 1994 |
Propionyl-L-carnitine prevents myocardial mechanical alterations due to pressure overload in rats.
Topics: Animals; Aorta; Cardiomegaly; Cardiotonic Agents; Carnitine; Constriction, Pathologic; Heart; Heart | 1994 |
Cardiac hypertrophy in juvenile visceral steatosis (jvs) mice with systemic carnitine deficiency.
Topics: Animals; Cardiomegaly; Carnitine; Cell Nucleus; Cytoplasm; DNA; Fatty Liver; Female; Homozygote; Mal | 1993 |
Assessment of the cardiostimulant action of propionyl-L-carnitine on chronically volume-overloaded rat hearts.
Topics: Animals; Carbon Dioxide; Cardiomegaly; Cardiotonic Agents; Carnitine; Mitochondria, Heart; Myocardia | 1993 |
Alterations in energy metabolism of hypertrophied rat cardiomyocytes: influence of propionyl-L-carnitine.
Topics: Adenosine Diphosphate; Adenosine Triphosphate; Animals; Cardiomegaly; Cardiotonic Agents; Carnitine; | 1995 |
Propionyl L-carnitine improvement of hypertrophied rat heart function is associated with an increase in cardiac efficiency.
Topics: Acetyl Coenzyme A; Acyl Coenzyme A; Adenosine Triphosphate; Animals; Cardiomegaly; Cardiotonic Agent | 1995 |
Effect of propionyl-L-carnitine on the mechanics of right and left papillary muscles from volume-overloaded rat hearts.
Topics: Anastomosis, Surgical; Animals; Aorta, Abdominal; Cardiomegaly; Cardiotonic Agents; Carnitine; Male; | 1996 |
[Total carnitine level in infants with cystic fibrosis and deficit supplementation by means of pharmacologic preparations and diet. Introductory remarks].
Topics: Cardiomegaly; Carnitine; Cystic Fibrosis; Enteral Nutrition; Food, Fortified; Hepatomegaly; Humans; | 1995 |
Control of oxidative metabolism in volume-overloaded rat hearts: effect of propionyl-L-carnitine.
Topics: Adenosine Diphosphate; Adenosine Triphosphate; Animals; Blood Pressure; Body Weight; Cardiomegaly; C | 1997 |
Altered expression of atrial natriuretic peptide and contractile protein genes in hypertrophied ventricle of JVS mice with systemic carnitine deficiency.
Topics: Actins; Animals; Atrial Natriuretic Factor; Body Weight; Cardiomegaly; Carnitine; Disease Models, An | 1997 |
A novel gene suppressed in the ventricle of carnitine-deficient juvenile visceral steatosis mice.
Topics: Amino Acid Sequence; Animals; Blotting, Northern; Brain; Cardiomegaly; Carnitine; Carnitine O-Palmit | 1997 |
Characteristics of cardiac hypertrophy in the juvenile visceral steatosis mouse with systemic carnitine deficiency.
Topics: Adenosine Diphosphate; Adenosine Monophosphate; Adenosine Triphosphate; Animals; Cardiomegaly; Carni | 1998 |
Cardiomegaly in the juvenile visceral steatosis (JVS) mouse is reduced with acute elevation of heart short-chain acyl-carnitine level after L-carnitine injection.
Topics: Aging; Animals; Body Weight; Cardiomegaly; Carnitine; Disease Models, Animal; Half-Life; Injections, | 1999 |
Catecholamine metabolism inhibitors and receptor blockades only partially suppress cardiac hypertrophy of juvenile visceral steatosis mice with systemic carnitine deficiency.
Topics: Adrenergic Antagonists; Animals; Atrial Natriuretic Factor; Body Weight; Cardiomegaly; Carnitine; Cy | 1999 |
Pyruvate dehydrogenase kinase 4 mRNA is increased in the hypertrophied ventricles of carnitine-deficient juvenile visceral steatosis (JVS) mice.
Topics: Amino Acid Sequence; Animals; Cardiomegaly; Carnitine; Gene Expression Regulation, Enzymologic; Hear | 1999 |
Milder childhood form of very long-chain acyl-CoA dehydrogenase deficiency in a 6-year-old Japanese boy.
Topics: Acyl-CoA Dehydrogenase, Long-Chain; Cardiomegaly; Carnitine; Child; Fatty Liver; Heart Failure; Hepa | 2000 |
Antioxidant changes in the hypertrophied heart due to energy metabolic disorder.
Topics: alpha-Tocopherol; Animals; Antioxidants; Blood Pressure; Cardiomegaly; Carnitine; Energy Metabolism; | 2001 |
Hereditary carnitine deficiency of muscle.
Topics: Aspartate Aminotransferases; Biopsy; Cardiomegaly; Carnitine; Child; Creatine Kinase; Genes, Recessi | 1975 |
Metabolism of lipids in experimental hypertrophic hearts of rabbits.
Topics: Animals; Cardiomegaly; Carnitine; Cholesterol; Fatty Acids; Fatty Acids, Nonesterified; Isoprotereno | 1979 |
Hemodynamic and metabolic activities of propionyl-L-carnitine in rats with pressure-overload cardiac hypertrophy.
Topics: Adenine Nucleotides; Analysis of Variance; Animals; Blood Pressure; Cardiac Output; Cardiomegaly; Ca | 1992 |
Functional and metabolic effects of propionyl-L-carnitine in the isolated perfused hypertrophied rat heart.
Topics: Animals; Blood Pressure; Cardiomegaly; Cardiotonic Agents; Carnitine; Energy Metabolism; Fatty Acids | 1992 |
Energy metabolism and mechanical function in perfused hearts of Syrian hamsters with dilated or hypertrophic cardiomyopathy.
Topics: Adenosine Triphosphate; Animals; Blood Pressure; Cardiomegaly; Carnitine; Coenzyme A; Coronary Circu | 1986 |
Mechanisms for altered carnitine content in hypertrophied rat hearts.
Topics: Animals; Aorta; Biological Transport, Active; Cardiomegaly; Carnitine; Ligation; Male; Perfusion; Ra | 1987 |
Experimental uremic cardiomyopathy--fact or fiction?
Topics: Animals; Carbachol; Cardiomegaly; Cardiomyopathies; Carnitine; Catecholamines; Isometric Contraction | 1986 |
Altered carnitine metabolism in spontaneously hypertensive rats.
Topics: Animals; Body Weight; Cardiomegaly; Carnitine; Hypertension; Liver; Male; Muscles; Myocardium; Organ | 1985 |
Defective lipid metabolism in the failing heart.
Topics: Animals; Carbon Isotopes; Cardiomegaly; Carnitine; Fatty Acids; Glucose; Guinea Pigs; Heart Failure; | 1968 |
Carnitine levels in human serum in health and disease.
Topics: Acetyl Coenzyme A; Acetyltransferases; Anorexia Nervosa; Benzoates; Carbon Radioisotopes; Cardiomega | 1974 |
Carnitine levels in human serum in health and disease.
Topics: Acetyl Coenzyme A; Acetyltransferases; Anorexia Nervosa; Benzoates; Carbon Radioisotopes; Cardiomega | 1974 |
Carnitine levels in human serum in health and disease.
Topics: Acetyl Coenzyme A; Acetyltransferases; Anorexia Nervosa; Benzoates; Carbon Radioisotopes; Cardiomega | 1974 |
Carnitine levels in human serum in health and disease.
Topics: Acetyl Coenzyme A; Acetyltransferases; Anorexia Nervosa; Benzoates; Carbon Radioisotopes; Cardiomega | 1974 |
Carnitine levels in human serum in health and disease.
Topics: Acetyl Coenzyme A; Acetyltransferases; Anorexia Nervosa; Benzoates; Carbon Radioisotopes; Cardiomega | 1974 |
Carnitine levels in human serum in health and disease.
Topics: Acetyl Coenzyme A; Acetyltransferases; Anorexia Nervosa; Benzoates; Carbon Radioisotopes; Cardiomega | 1974 |
Carnitine levels in human serum in health and disease.
Topics: Acetyl Coenzyme A; Acetyltransferases; Anorexia Nervosa; Benzoates; Carbon Radioisotopes; Cardiomega | 1974 |
Carnitine levels in human serum in health and disease.
Topics: Acetyl Coenzyme A; Acetyltransferases; Anorexia Nervosa; Benzoates; Carbon Radioisotopes; Cardiomega | 1974 |
Carnitine levels in human serum in health and disease.
Topics: Acetyl Coenzyme A; Acetyltransferases; Anorexia Nervosa; Benzoates; Carbon Radioisotopes; Cardiomega | 1974 |