Glutamate Regulates Dystrophin-71 levels in Glia Cells

Galaz-Vega, Roque; Hernández‐Kelly, Luisa C.; Méndez, José Alfredo Tirado; Cisneros, Bulmaro; Ortega, Arturo

doi:10.1007/s11064-004-2446-6

Cited by 10 publications

(10 citation statements)

References 23 publications

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“…The results of the study of Golaszewski et al indicate impaired cortical plasticity in glutamate-dependent excitation circuits in mentally retarded BMD patients consistent with the results of several experimental studies indicating abnormal glutamatergic transmission in muscle diseases caused by mutations within the dystrophin-encoding gene [9]. In particular, the product of the dystrophin gene, dystrophin-71, in glutamate receptor signaling and possibly clustering, appears to be involved [27]. Besides, dystrophin-deficient mdx mice are more resistant to kainic acid-induced seizures but not to GABA antagonist-induced seizures compared with control mice.…”

Section: Discussionsupporting

confidence: 61%

Electrophysiological Assessment of CNS Abnormalities in Muscular Dystrophy

Golaszewski¹,

Nardone²

2019

Muscular Dystrophies

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Patients affected by muscular dystrophies often show CNS abnormalities. Patients with dystrophinopathies exhibit intellectual disabilities and mental retardation, while subjects with facioscapulohumeral muscular dystrophy (FSHD) often show epilepsy. Dystrophin and associated proteins have important roles in the CNS. Many patients with Duchenne and Becker muscular dystrophies (DMD/ BMD) have cognitive impairment, learning disability, and variable degrees of mental retardation in addition to progressive muscular weakness. Unfortunately, the assessment of cortical function with TMS in DMD patients has not been able to delineate a clear picture and has yielded contradictory results. No TMS studies have been performed on BMD patients. Repetitive transcranial magnetic stimulation (rTMS) modulates cortical excitability, possibly by inducing a short-term increase in synaptic efficacy, and can be used to investigate motor cortex excitability in BMD patients. Changes in the size and threshold of motor evoked potentials (MEPs) and cortical silent period (CSP) duration evoked by rTMS delivered in 5 Hz trains of stimuli at suprathreshold intensity can be tested. Impaired muscular function might be partially compensated by an enhancement of motor excitability at the cortical level and/or at α-motoneuron level. TMS may thus offer a reliable means to characterize also important neurophysiologic and pathophysiologic aspects of cortical involvement in muscular dystrophy.

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Section: Discussionsupporting

confidence: 61%

Electrophysiological Assessment of CNS Abnormalities in Muscular Dystrophy

Golaszewski¹,

Nardone²

2019

Muscular Dystrophies

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“…Astrocytic endfeet surrounding brain blood vessels exhibit prominent Dp71 expression (Fig. 2) along with β-DG, syntrophin isoforms, and α-DB1 [26,31,[182][183][184]. High-resolution immunoelectron microscopy studies have demonstrated that AQP4 and the inwardly rectifying K + channel, Kir4.1, are localized selectively in the astrocytic membrane that is in direct contact with the basal lamina facing the blood vessel [179,[185][186][187].…”

Section: Bbb and Cpmentioning

confidence: 99%

Role of dystrophin and utrophin for assembly and function of the dystrophin glycoprotein complex in non-muscle tissue

Haenggi

Fritschy

2006

Cell. Mol. Life Sci.

148

112

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The dystrophin glycoprotein complex (DGC) is a multimeric protein assembly associated with either the X-linked cytoskeletal protein dystrophin or its autosomal homologue utrophin. In striated muscle cells, the DGC links the extracellular matrix to the actin cytoskeleton and mediates three major functions: structural stability of the plasma membrane, ion homeostasis, and transmembrane signaling. Mutations affecting the DGC underlie major forms of congenital muscle dystrophies. The DGC is prominent also in the central and peripheral nervous system and in tissues with a secretory function or which form barriers between functional compartments, such as the blood-brain barrier, choroid plexus, or kidney. A considerable molecular heterogeneity arises from cell-specific expression of its constituent proteins, notably short C-terminal isoforms of dystrophin. Experimentally, the generation of mice carrying targeted gene deletions affecting the DGC has clarified the interdependence of DGC proteins for assembly of the complex and revealed its importance for brain development and regulation of the 'milieu intérieur. Here, we focus on recent studies of the DGC in brain, blood-brain barrier and choroid plexus, retina, and kidney and discuss the role of dystrophin isoforms and utrophin for assembly of the complex in these tissues.

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“…Com o tamanho total de 427kDa (kilodalton) e conhecida como Dp427, a distrofina apresenta isoformas distribuídas diferentemente nos tecidos, como a Dp427c nas estruturas cerebrais e Dp427p nas células de Purkinje (GALAZ-VEGA et al, 2005;DAOUD et al, 2009). Importante salientar que embora a distrofina seja encontrada em maior concentração na junção neuromuscular, a proteína foi encontrada em quantidades semelhantes no encéfalo (incluindo cérebro e córtex cerebelar) (LIDOV et al, 1990;CYRULNIK;HINTON, 2008).…”

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<b>Estudo morfológico do Sistema Nervoso Central de cães com Distrofia Muscular do Golden Retriever (GRMD)</b>

Guimarães¹

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Em primeiro lugar, e mais importante, agradeço a Deus, pois nunca imaginei estar onde estou, demorei a entender os planos dEle para minha vida, mas hoje posso ver que Ele tem grandes planos para mim, exatamente onde estou, e sou muito grata a Ele por isso! Aos meus pais, por todo esforço e dedicação, por todo apoio emocional, financeiro, por me educarem com amor, esforço, e por serem os velhinhos mais lindos da minha vida. Amo vocês, e agradeço a Deus a cada dia por ter nascido de vocês, pois vocês são os melhores e mais maravilhosos pais que eu poderia ter. A minha linda avó Silvina, a mulher mais guerreira que conheci na vida, e nunca conhecerei alguém com tanta garra e determinação feito ela. A senhora é meu exemplo de força, garra e amor pela família! É um privilégio imenso poder conviver ao seu lado até aqui. E vamos, rumo aos 100 aninhos vó! À minha orientadora, Patrícia Braga, muito obrigada por tudo! Por confiar esse trabalho a mim, por acreditar no meu potencial, por sempre me estimular, pelos ensinamentos e pelo carinho que sempre teve por mim. Esses dois anos não foram nada fáceis para nós, e sei que para você foi muito difícil, mas acredito que fizemos o nosso melhor nesse trabalho, e uma com a outra. Espero que possamos estar mais próximas daqui para frente, e que eu possa aproveitar muito mais tudo o que você tem para me ensinar, muito mais mesmo. Aos meus familiares que de alguma forma participaram, e tiveram que me aturar nessa jornada, amo vocês...minhas irmãs, Issinha que me ouviu, e ouviu, e ouviu, e me abrigou na sua casa, junto com meu cunhadinho Gezinho, muito obrigada, a Érika que me ofereceu apoio sempre. Aos ibitinguenses, que me aguentaram de perto e de longe. Aos meus tios queridos, Rosana e Misael, sempre preocupados em saber o andamento, oferecendo apoio, carinho. Aos meus amigos, sempre preocupados em ajudar, apoiar, dar força...vocês tornam minha vida mais leve, obrigada por isso

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Glutamate Regulates Dystrophin-71 levels in Glia Cells

Cited by 10 publications

References 23 publications

Electrophysiological Assessment of CNS Abnormalities in Muscular Dystrophy

Electrophysiological Assessment of CNS Abnormalities in Muscular Dystrophy

Role of dystrophin and utrophin for assembly and function of the dystrophin glycoprotein complex in non-muscle tissue

<b>Estudo morfológico do Sistema Nervoso Central de cães com Distrofia Muscular do Golden Retriever (GRMD)</b>

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