MELAS and L-arginine therapy: pathophysiology of stroke-like episodes

Annals of the New York Academy of Sciences
Yasutoshi KogaT Matsuishi

Abstract

Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is a maternally inherited multisystem mitochondrial disorder. Although many molecular and cellular mechanisms have been discovered leading to mitochondrial cytopathy, the pathogenic mechanism of stroke-like episodes seen in MELAS has not been clarified yet. According to the muscle and brain pathology and vascular physiology, mitochondrial angiopathy, or endothelial dysfunction, were proposed to play an important role for developing stroke-like episodes. Based on a hypothesis of mitochondrial angiopathy theory, we use L-arginine in MELAS patients and report its usefulness. This review aims to give a general idea on the actual knowledge about the possible pathogenic mechanism of stroke-like episodes, including clinical symptoms that lead to stroke-like episodes, muscle, or brain pathology, molecular cellular functions, neuroimagings including MRI, MRS, and SPECT, and the proposed site of action of L-arginine therapy on MELAS patients. Currently, L-arginine therapy may be the most promising for the treatment of stroke-like episodes in MELAS.

References

Jun 1, 1989·Annals of Neurology·R Sakuta, I Nonaka
Dec 1, 1988·Annals of Neurology·Y KogaK Nihei
Jan 29, 2000·Acta Neuropathologica·C TanahashiY Hashizume
Nov 9, 2000·Neuroradiology·T OhshitaS Nakamura
Jul 21, 2001·Journal of Neuroimaging : Official Journal of the American Society of Neuroimaging·T NariaiM Senda
Mar 1, 2003·Neuromuscular Disorders : NMD·Atsuko KogaToyojiro Matsuishi
Sep 8, 2004·Brain & Development·Masaya KubotaMariko Momoi-Yoshida
Feb 25, 2005·Neurology·Y KogaToyojiro Matsuishi
Mar 12, 2005·Journal of the Neurological Sciences·Ali NainiEric A Schon
Jul 5, 2005·Pediatric Neurology·Atsuko NoguchiGoro Takada
Oct 6, 2005·Mutation Research·Roshan S Jahangir TafrechiAnton K Raap
Aug 1, 2007·Brain & Development·Junko NishiokaYasutoshi Koga
Feb 22, 2008·Brain & Development·Hiromichi ItoShoji Kagami
Jul 22, 2008·Clinical Nutrition : Official Journal of the European Society of Parenteral and Enteral Nutrition·David ComanAvihu Boneh
Aug 2, 2008·Journal of Neurology·S Stoquart-ElsankariO Godefroy
Oct 14, 2008·Revue neurologique·F MoutaouakilI Slassi
Dec 19, 2008·Journal of the Neurological Sciences·Takahiro IizukaFumihiko Sakai
May 23, 2009·Mitochondrion·Masamichi IkawaMakoto Yoneda

❮ Previous
Next ❯

Citations

May 17, 2013·Pediatric Radiology·Tadahiro MitaniHitoshi Osaka
Apr 25, 2012·Current Treatment Options in Cardiovascular Medicine·Hurmina Muqtadar, Fernando D Testai
Jan 29, 2013·Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics·Amy Goldstein, Lynne A Wolfe
Jan 31, 2013·Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics·Douglas S Kerr
May 12, 2012·Brain : a Journal of Neurology·Nichola Z LaxDoug M Turnbull
Mar 16, 2012·Journal of Neuro-ophthalmology : the Official Journal of the North American Neuro-Ophthalmology Society·Seo Young ChoiJi Soo Kim
Dec 25, 2012·International Journal of Molecular Sciences·Juliana GambaCelia H Tengan
Jul 19, 2012·World Journal of Gastroenterology : WJG·Elisa FabbriUmberto Volta
Apr 23, 2013·Clinical Nuclear Medicine·Izzie Jacques NamerChristian Marescaux
Apr 27, 2013·Journal of Neurology, Neurosurgery, and Psychiatry·Pankaj SharmaJames F Meschia
May 6, 2014·Revue neurologique·V ProcaccioP Reynier
Dec 3, 2014·Molecular Genetics and Metabolism·Mauro ScarpelliMassimiliano Filosto
Nov 16, 2013·Seminars in Neurology·Dulanji K Kuruppu, Brandy R Matthews
Dec 14, 2011·Expert Opinion on Drug Metabolism & Toxicology·Josef Finsterer, Sinda Zarrouk Mahjoub
Dec 19, 2012·Seminars in Pediatric Neurology·Suzanne Debrosse, Sumit Parikh
Apr 21, 2012·Brain & Development·Hsu-Ling YehYau-Huei Wei
Nov 15, 2011·Brain & Development·Masafumi Matsuo
Jun 17, 2011·Seminars in Fetal & Neonatal Medicine·Anu Suomalainen
Mar 30, 2011·Biochimica Et Biophysica Acta·Shuichi YatsugaUNKNOWN Taro Matsuoka for MELAS Study Group in Japan
Dec 18, 2015·BBA Clinical·Eva TerniMichelangelo Mancuso
Jul 15, 2015·Epilepsy & Behavior : E&B·Shamima Rahman
Jul 4, 2012·British Journal of Pharmacology·Juan Garrido-MaraverJosé A Sánchez-Alcázar
Nov 15, 2012·The EMBO Journal·Werner J H KoopmanPeter H G M Willems
Oct 15, 2013·British Journal of Pharmacology·M KanabusS Rahman
Sep 19, 2012·Molecular and Cellular Neurosciences·Sarah PearceAntonella Spinazzola
Sep 26, 2013·Biotechnic & Histochemistry : Official Publication of the Biological Stain Commission·Y KanazawaH Fujino
Apr 15, 2016·Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·Sung-Hee KimJi-Soo Kim
Jun 12, 2016·Neuropathology and Applied Neurobiology·N Z LaxD M Turnbull
Aug 2, 2016·Current Pain and Headache Reports·Konark Malhotra, David S Liebeskind
Jul 16, 2016·Acta neurologica Belgica·Guillaume TaiebDimitri Renard
Apr 21, 2017·Tropical Doctor·Josef Finsterer, Sinda Zarrouk-Mahjoub
Jan 5, 2019·Clinical Pediatrics·Kallol K SetRajkumar Agarwal
Feb 9, 2019·Journal of Inherited Metabolic Disease·Alfonso Oyarzabal, Isaac Marin-Valencia
May 24, 2020·Annals of Neurology·Cesar A P F AlvesGiulio Zuccoli
Oct 1, 2018·Journal of Neurology·Yasutoshi KogaKatsunori Fujii

❮ Previous
Next ❯

Related Concepts

Related Feeds

Acute Stroke

A stroke occurs when blood supply to the brain is interrupted depriving the brain of oxygen and nutrients. This feed focuses cerebrovascular accidents including ischemic and paralytic stroke.