The serum nitric oxide levels in patients with Duchenne muscular dystrophy

Gücüyener, Kıvılcım; Ergenekon, Ebru; Erbaş, Deniz; Pınarlı, Faruk Güçlü; Serdaroğlu, Ayşe

doi:10.1016/s0387-7604(00)00106-6

Cited by 30 publications

(29 citation statements)

References 9 publications

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“…Some have proposed that improper control of the vasculature results from the reduced levels of membrane-associated nNOS in DMD muscles [26], and this reduction may lead to eventual muscle damage [10]. It has been reported that serum NO levels of DMD individuals were significantly lower than those of control and cerebral palsy groups [16]. Our results, which include data from other neuromuscular diseases, reaffirm these findings.…”

Section: Discussionsupporting

confidence: 87%

Decreased Total Nitric Oxide Production in Patients with Duchenne Muscular Dystrophy

et al. 2004

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Plasma nitric oxide (NO) levels in Duchenne muscular dystrophy (DMD) patients were significantly lower than those observed in both healthy controls and in patients with other neuromuscular disorders. The correlation between NO level and ejection fraction was significant (r = –0.384, p = 0.0391) in the DMD group. Disruption of NO systems may contribute to the development of muscular dystrophy and have implications for therapeutic strategies.

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Section: Discussionsupporting

confidence: 87%

Decreased Total Nitric Oxide Production in Patients with Duchenne Muscular Dystrophy

et al. 2004

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“…This hypothesis was based on observations from patients with Duchenne muscular dystrophy (DMD) where mutations in the dystrophin gene result in an uncoupling of nNOS from the contractile apparatus, leading to the loss of contractile function seen in DMD, causing low serum nitric oxide [86][87][88]. While further work is required to elucidate the mechanism regulating NOS activity, an uncoupling of NOS from dynein ATPase seems unlikely as PCD phenotypes with hyper-frequent or motile dyskinetic cilia have low nitric oxide similar to patients with static cilia.…”

Section: Decreased Expression Of Nos Isoenzymesmentioning

confidence: 99%

Nitric oxide in primary ciliary dyskinesia

et al. 2012

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Nitric oxide is continually synthesised in the respiratory epithelium and is upregulated in response to infection or inflammation. Primary ciliary dyskinesia (PCD) is characterised by recurrent sinopulmonary infections due to impaired mucociliary clearance. Despite chronic infections, nasal nitric oxide in such patients is markedly reduced and is used as a screening test for this condition. These low levels were first described .15 yrs ago but the underlying mechanisms have yet to be fully elucidated. We review epithelial nitric oxide synthesis, release and measurement in the upper airways with particular reference to PCD. The key hypotheses that have been proposed to explain the low nitric oxide levels in this condition are explored and the potential benefits of augmenting airway nitric oxide levels are considered. Further work in these patients clarifying both whether the respiratory epithelium is able to biosynthesise normal levels of nitric oxide and the role played by abnormalities in the anatomy of the paranasal sinuses is essential. While nitric oxide augmentation is unlikely to be beneficial in common PCD phenotypes, it has potential in the treatment of secondary dyskinesias and may also improve treatment of bacterial infections, particularly where biofilms are implicated.

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“…While these more general cellular functions are relevant for NOS involvement in the progression of DMD [67], there is also evidence for a more specialized role of NOS in modulating UGC function at the NMJ. NO also acts as both an antero- and retrograde modulator of synaptic transmission at central synapses [68].…”

Section: Roles Of the Dgc At The Neuromuscular Junctionmentioning

confidence: 99%

The Roles of the Dystrophin-Associated Glycoprotein Complex at the Synapse

et al. 2009

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Duchenne muscular dystrophy is caused by mutations in the dystrophin gene and is characterized by progressive muscle wasting. A number of Duchenne patients also present with mental retardation. The dystrophin protein is part of the highly conserved dystrophin-associated glycoprotein complex (DGC) which accumulates at the neuromuscular junction (NMJ) and at a variety of synapses in the peripheral and central nervous systems. Many years of research into the roles of the DGC in muscle have revealed its structural function in stabilizing the sarcolemma. In addition, the DGC also acts as a scaffold for various signaling pathways. Here, we discuss recent advances in understanding DGC roles in the nervous system, gained from studies in both vertebrate and invertebrate model systems. From these studies, it has become clear that the DGC is important for the maturation of neurotransmitter receptor complexes and for the regulation of neurotransmitter release at the NMJ and central synapses. Furthermore, roles for the DGC have been established in consolidation of long-term spatial and recognition memory. The challenges ahead include the integration of the behavioral and mechanistic studies and the use of this information to identify therapeutic targets.

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The serum nitric oxide levels in patients with Duchenne muscular dystrophy

Cited by 30 publications

References 9 publications

Decreased Total Nitric Oxide Production in Patients with Duchenne Muscular Dystrophy

Decreased Total Nitric Oxide Production in Patients with Duchenne Muscular Dystrophy

Nitric oxide in primary ciliary dyskinesia

The Roles of the Dystrophin-Associated Glycoprotein Complex at the Synapse

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