MELAS and l-arginine therapy

Koga, Yasutoshi; Akita, Yukihiro; Nishioka, Junko; Yatsuga, Shuichi; Povalko, Nataliya; Katayama, Koujyu; Matsuishi, Toyojiro

doi:10.1016/j.mito.2006.11.006

Cited by 108 publications

(75 citation statements)

References 40 publications

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“…Unblinded studies have showed that L-arginine therapy can be beneficial in stroke-like episode treatment and prevention. Intravenous L-arginine infusion during the acute phase has been shown to improve stroke-like episode symptoms whereas oral L-arginine supplementation during the interictal phase has been shown to decrease the frequency and severity of stroke-like episodes [48,59]. The suggested intravenous L-arginine dose is 0.5 g/kg for children or 10 g/m 2 body surface area for adults with a similar daily dose to be given orally in three divided doses during the interictal phase [58].…”

Section: Management Of Complicationsmentioning

confidence: 99%

MELAS syndrome: Clinical manifestations, pathogenesis, and treatment options

El‐Hattab

Adesina

Jones

et al. 2015

Molecular Genetics and Metabolism

444

438

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Section: Management Of Complicationsmentioning

confidence: 99%

MELAS syndrome: Clinical manifestations, pathogenesis, and treatment options

El‐Hattab

Adesina

Jones

et al. 2015

Molecular Genetics and Metabolism

444

438

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“…L-arginine is suitable for oral administration because it is hydrophilic. The therapeutic effects of L-arginine were investigated in patients with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) [61]. During the acute phase of MELAS, L-arginine concentrations were significantly lower in patients than in control subjects.…”

Section: Mitochondria and Reactive Oxygen Speciesmentioning

confidence: 99%

Mitochondrial drug delivery systems for macromolecule and their therapeutic application to mitochondrial diseases

Yamada

Harashima

2008

Advanced Drug Delivery Reviews

222

148

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Key words: Mitochondria, mitochondrial drug delivery, mitochondrial macromolecule delivery, MITO-Porter, membrane fusion, multifunctional envelope-type nano device (MEND), mitochondrial protein therapy, mitochondrial gene therapy, mitochondrial protein import machinery, mitochondrial DNA. Yamada, Y., et al. AbstractMitochondrial dysfunction has been implicated in a variety of human disorders-the so-called mitochondrial diseases. Therefore, the organelle is a promising therapeutic drug target. In this review, we describe the key role of mitochondria in living cells, a number of mitochondrial drug delivery systems and mitochondria-targeted therapeutic strategies. In particular, we discuss mitochondrial delivery of macromolecules, such as proteins and nucleic acids. The discussion of protein delivery is limited primarily to the mitochondrial import machinery. In the section on mitochondrial gene delivery and therapy, we discuss mitochondrial diseases caused by mutations in mitochondrial DNA, several gene delivery strategies and approaches to mitochondrial gene therapy. This review also summarizes our current efforts regarding liposome-based delivery system including use of a multifunctional envelope-type nano device (MEND) and mitochondrial liposome-based delivery as anti-cancer therapies. Furthermore, we introduce the novel MITO-Porter-a liposome-based mitochondrial delivery system that functions using a membrane-fusion mechanism.Yamada, Y., et al. 3

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“…Therefore, arginine and citrulline can be of therapeutic utility in treating NO deficiency-related manifestations of mitochondrial diseases [25]. Arginine supplementation to individuals with MELAS syndrome resulted in an improvement in clinical symptoms associated with stroke-like episodes and a decrease in the frequency and severity of these episodes [47]. However, there are no clinical studies evaluating the effect of arginine or citrulline supplementation on other mitochondrial diseases.…”

Section: Treatment Options For Mitochondrial Diseasesmentioning

confidence: 99%

Mitochondrial cytopathies

El‐Hattab

Scaglia

2016

Cell Calcium

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Mitochondria are found in all nucleated human cells and perform a variety of essential functions, including the generation of cellular energy. Most of mitochondrial proteins are encoded by the nuclear DNA (nDNA) whereas a very small fraction is encoded by the mitochondrial DNA (mtDNA). Mutations in mtDNA or mitochondria-related nDNA genes can result in mitochondrial dysfunction which leads to a wide range of cellular perturbations including aberrant calcium homeostasis, excessive reactive oxygen species production, dysregulated apoptosis, and insufficient energy generation to meet the needs of various organs, particularly those with high energy demand. Impaired mitochondrial function in various tissues and organs results in the multi-organ manifestations of mitochondrial diseases including epilepsy, intellectual disability, skeletal and cardiac myopathies, hepatopathies, endocrinopathies, and nephropathies. Defects in nDNA genes can be inherited in an autosomal or X-linked manners, whereas, mtDNA is maternally inherited. Mitochondrial diseases can result from mutations of nDNA genes encoding subunits of the electron transport chain complexes or their assembly factors, proteins associated with the mitochondrial import or networking, mitochondrial translation factors, or proteins involved in mtDNA maintenance. MtDNA defects can be either point mutations or rearrangements. The diagnosis of mitochondrial disorders can be challenging in many cases and is based on clinical recognition, biochemical screening, histopathological studies, functional studies, and molecular genetic testing. Currently, there are no satisfactory therapies available for mitochondrial disorders that significantly alter the course of the disease. Therapeutic options include symptomatic treatment, cofactor supplementation, and exercise.

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MELAS and l-arginine therapy

Cited by 108 publications

References 40 publications

MELAS syndrome: Clinical manifestations, pathogenesis, and treatment options

MELAS syndrome: Clinical manifestations, pathogenesis, and treatment options

Mitochondrial drug delivery systems for macromolecule and their therapeutic application to mitochondrial diseases

Mitochondrial cytopathies

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