Creatine Therapy in Myophosphorylase Deficiency (McArdle Disease)

Abstract

This is the first controlled study indicating that creatine supplementation improves skeletal muscle function in McArdle disease.

Creatine has no beneficial effect on skeletal muscle energy metabolism in patients with single mitochondrial DNA deletions: a placebo‐controlled, double‐blind 31P‐MRS crossover study

“…The failure to improve muscle energy metabolism in our patients with mitochondrial disorders may therefore be attributed to the inefficacy of Cr supplementation to increase intramuscular PCr contents. This finding is in agreement with recently published results in patients with myotonic dystrophy type 1, muscular dystrophy and McArdle disease (Vorgerd et al. , 2000a, 2002; Zange et al.…”

“…In our series of patients, Cr supplementation caused a significant increase in plasma concentrations of Cr and was well-tolerated without major side-effects. This finding is in line with previous reports showing that Cr ingestion at a dosage up to 20 g/day is safe in patients with various neuromuscular disorders (Tarnopolsky et al, 1997(Tarnopolsky et al, , 2004Tarnopolsky and Martin, 1999a;Klopstock et al, 2000;Vorgerd et al, 2000a;Walter et al, 2000Walter et al, , 2002Vorgerd et al, 2002).…”

Creatine has no beneficial effect on skeletal muscle energy metabolism in patients with single mitochondrial DNA deletions: a placebo‐controlled, double‐blind 31P‐MRS crossover study

“…The failure to improve muscle energy metabolism in our patients with mitochondrial disorders may therefore be attributed to the inefficacy of Cr supplementation to increase intramuscular PCr contents. This finding is in agreement with recently published results in patients with myotonic dystrophy type 1, muscular dystrophy and McArdle disease (Vorgerd et al. , 2000a, 2002; Zange et al.…”

“…In our series of patients, Cr supplementation caused a significant increase in plasma concentrations of Cr and was well-tolerated without major side-effects. This finding is in line with previous reports showing that Cr ingestion at a dosage up to 20 g/day is safe in patients with various neuromuscular disorders (Tarnopolsky et al, 1997(Tarnopolsky et al, , 2004Tarnopolsky and Martin, 1999a;Klopstock et al, 2000;Vorgerd et al, 2000a;Walter et al, 2000Walter et al, , 2002Vorgerd et al, 2002).…”

Creatine as an antioxidant

“…These observations lend further support to the concept that the beneficial effects of Cr that are being observed at the therapeutic and preventive levels (Sipila et al 1981;Osbakken et al 1992;Gordon et al 1995;Heinanen et al 1999;Matthews et al 1999;Vorgerd et al 2000;Hespel et al 2001;Mazzini et al 2001;Stout et al 2001;Witte et al 2001;Tarnopolsky et al 2004;Hansen et al 2006;Kuethe et al 2006) may not depend exclusively on the role of Cr in cellular energetics, but rather on multiple positive interactions with diverse cellular targets (Lawler et al 2002;Louis et al 2004;Lenz et al 2005;Alfieri et al 2006;Olsen et al 2006;Sestili et al 2006;Bender et al 2007;Brosnan et al 2007;Ducray et al 2007;Rodriguez et al 2007;Guidi et al 2008;Oliveira et al 2008). The comprehension of Cr pleiotropism is an emerging issue in research literature, and the results in this direction might be important to rationally interpret the role and exploit the therapeutic/preventive potential of Cr supplementation in specific situations.…”

Effects of creatine supplementation and exercise training on fitness in men 55–75 yr old

“…The finding that CR can stimulate the beneficial effects of resistance training on muscle volume and functional capacity, at least in young healthy subjects (5,27,34,48,50), is interesting in the context of the preservation of muscle functional capacity in the elderly. Furthermore, consistent with the observations in young healthy subjects, CR has been found to enhance muscular functional capacity in patients afflicted by neuromuscular diseases (45,51,52) as well as in cardiac patients (11,22). It has been suggested that older people might respond better to CR than young people, because they may have a lower muscle Cr content (46).…”

Creatine has no beneficial effect on skeletal muscle energy metabolism in patients with single mitochondrial DNA deletions: a placebo‐controlled, double‐blind 31P‐MRS crossover study

“…The failure to improve muscle energy metabolism in our patients with mitochondrial disorders may therefore be attributed to the inefficacy of Cr supplementation to increase intramuscular PCr contents. This finding is in agreement with recently published results in patients with myotonic dystrophy type 1, muscular dystrophy and McArdle disease (Vorgerd et al. , 2000a, 2002; Zange et al.…”

“…In our series of patients, Cr supplementation caused a significant increase in plasma concentrations of Cr and was well-tolerated without major side-effects. This finding is in line with previous reports showing that Cr ingestion at a dosage up to 20 g/day is safe in patients with various neuromuscular disorders (Tarnopolsky et al, 1997(Tarnopolsky et al, , 2004Tarnopolsky and Martin, 1999a;Klopstock et al, 2000;Vorgerd et al, 2000a;Walter et al, 2000Walter et al, , 2002Vorgerd et al, 2002).…”

Creatine as an antioxidant

“…These observations lend further support to the concept that the beneficial effects of Cr that are being observed at the therapeutic and preventive levels (Sipila et al 1981;Osbakken et al 1992;Gordon et al 1995;Heinanen et al 1999;Matthews et al 1999;Vorgerd et al 2000;Hespel et al 2001;Mazzini et al 2001;Stout et al 2001;Witte et al 2001;Tarnopolsky et al 2004;Hansen et al 2006;Kuethe et al 2006) may not depend exclusively on the role of Cr in cellular energetics, but rather on multiple positive interactions with diverse cellular targets (Lawler et al 2002;Louis et al 2004;Lenz et al 2005;Alfieri et al 2006;Olsen et al 2006;Sestili et al 2006;Bender et al 2007;Brosnan et al 2007;Ducray et al 2007;Rodriguez et al 2007;Guidi et al 2008;Oliveira et al 2008). The comprehension of Cr pleiotropism is an emerging issue in research literature, and the results in this direction might be important to rationally interpret the role and exploit the therapeutic/preventive potential of Cr supplementation in specific situations.…”

Effects of creatine supplementation and exercise training on fitness in men 55–75 yr old

“…The finding that CR can stimulate the beneficial effects of resistance training on muscle volume and functional capacity, at least in young healthy subjects (5,27,34,48,50), is interesting in the context of the preservation of muscle functional capacity in the elderly. Furthermore, consistent with the observations in young healthy subjects, CR has been found to enhance muscular functional capacity in patients afflicted by neuromuscular diseases (45,51,52) as well as in cardiac patients (11,22). It has been suggested that older people might respond better to CR than young people, because they may have a lower muscle Cr content (46).…”

Creatine has no beneficial effect on skeletal muscle energy metabolism in patients with single mitochondrial DNA deletions: a placebo‐controlled, double‐blind 31P‐MRS crossover study

“…The failure to improve muscle energy metabolism in our patients with mitochondrial disorders may therefore be attributed to the inefficacy of Cr supplementation to increase intramuscular PCr contents. This finding is in agreement with recently published results in patients with myotonic dystrophy type 1, muscular dystrophy and McArdle disease (Vorgerd et al. , 2000a, 2002; Zange et al.…”

“…In our series of patients, Cr supplementation caused a significant increase in plasma concentrations of Cr and was well-tolerated without major side-effects. This finding is in line with previous reports showing that Cr ingestion at a dosage up to 20 g/day is safe in patients with various neuromuscular disorders (Tarnopolsky et al, 1997(Tarnopolsky et al, , 2004Tarnopolsky and Martin, 1999a;Klopstock et al, 2000;Vorgerd et al, 2000a;Walter et al, 2000Walter et al, , 2002Vorgerd et al, 2002).…”

Creatine as an antioxidant

“…These observations lend further support to the concept that the beneficial effects of Cr that are being observed at the therapeutic and preventive levels (Sipila et al 1981;Osbakken et al 1992;Gordon et al 1995;Heinanen et al 1999;Matthews et al 1999;Vorgerd et al 2000;Hespel et al 2001;Mazzini et al 2001;Stout et al 2001;Witte et al 2001;Tarnopolsky et al 2004;Hansen et al 2006;Kuethe et al 2006) may not depend exclusively on the role of Cr in cellular energetics, but rather on multiple positive interactions with diverse cellular targets (Lawler et al 2002;Louis et al 2004;Lenz et al 2005;Alfieri et al 2006;Olsen et al 2006;Sestili et al 2006;Bender et al 2007;Brosnan et al 2007;Ducray et al 2007;Rodriguez et al 2007;Guidi et al 2008;Oliveira et al 2008). The comprehension of Cr pleiotropism is an emerging issue in research literature, and the results in this direction might be important to rationally interpret the role and exploit the therapeutic/preventive potential of Cr supplementation in specific situations.…”

Effects of creatine supplementation and exercise training on fitness in men 55–75 yr old

“…The finding that CR can stimulate the beneficial effects of resistance training on muscle volume and functional capacity, at least in young healthy subjects (5,27,34,48,50), is interesting in the context of the preservation of muscle functional capacity in the elderly. Furthermore, consistent with the observations in young healthy subjects, CR has been found to enhance muscular functional capacity in patients afflicted by neuromuscular diseases (45,51,52) as well as in cardiac patients (11,22). It has been suggested that older people might respond better to CR than young people, because they may have a lower muscle Cr content (46).…”