Unique Role of Dystroglycan in Peripheral Nerve Myelination, Nodal Structure, and Sodium Channel Stabilization

Saito, Fumiaki; Moore, Steven A.; Barresi, Rita; Henry, Michael D.; Messing, Albee; Ross-Barta, Susan E.; Cohn, Ronald D.; Williamson, Roger A.; Sluka, Kathleen A.; Sherman, Diane L.; Brophy, Peter; Schmelzer, James D.; Low, Phillip A.; Wrabetz, Lawrence; Feltri, M. Laura; Campbell, Kevin P.

doi:10.1016/s0896-6273(03)00301-5

Cited by 223 publications

(239 citation statements)

References 61 publications

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“…[38][39][40] Admittedly, loss of the entire dystroglycan protein might have effects beyond those caused by defects in its glycosylation, but these data nevertheless support the possibility that all glycosylation defects in the dystroglycanopathies could be centered on dystroglycan. This characterization represents the current state of the literature.…”

Section: Glycosylation Of Dystroglycanmentioning

confidence: 89%

Mechanisms of Disease: congenital muscular dystrophies—glycosylation takes center stage

Martin

2006

Nat Rev Neurol

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SUMMARYRecent studies have defined a group of muscular dystrophies, now termed the dystroglycanopathies, as novel disorders of glycosylation. These conditions include Walker-Warburg syndrome, muscleeye-brain disease, Fukuyama-type congenital muscular dystrophy, congenital muscular dystrophy types 1C and 1D, and limb-girdle muscular dystrophy type 2I. Although clinical findings can be highly variable, dystroglycanopathies are all characterized by cortical malformations and ocular defects at the more severe end of the clinical spectrum, in addition to muscular dystrophy. All of these disorders are defined by the underglycosylation of α-dystroglycan. Defective glycosylation of dystroglycan severs the link between this important cell adhesion molecule and the extracellular matrix, thereby contributing to cellular pathology. Recent experiments indicate that glycosylation might not only define forms of muscular dystrophy but also provide an avenue to the development of therapies for these disorders.

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Section: Glycosylation Of Dystroglycanmentioning

confidence: 89%

Mechanisms of Disease: congenital muscular dystrophies—glycosylation takes center stage

Martin

2006

Nat Rev Neurol

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“…Von Frey filaments of varying bending forces (1.5, 0.7, 0.3, 0.2, 0.08 mN) were applied 10 times to the each plantar surface of the hindpaw and the number of withdrawals was recorded. 36 The mechanical withdrawal threshold was measured before the first run (day -1), before injection 1 (day 0), before the third run (day 4), before injection 2 (day 5), and 24 hours after the second injection (day 6) (Fig 1). In the current study, we interpreted an increase in the number of withdrawals as cutaneous hyperalgesia of the paw.…”

Section: Mechanical Withdrawal Response Of the Pawmentioning

confidence: 99%

Muscle Fatigue Increases the Probability of Developing Hyperalgesia in Mice

Yokoyama

Lisi

Moore

et al. 2007

The Journal of Pain

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Chronic muscle pain is a major clinical problem that is often associated with fatigue. Conversely, chronic fatigue conditions are commonly associated with muscle pain. We tested the hypothesis that muscle fatigue enhances hyperalgesia associated with injection of acidic saline into muscle. We evaluated mechanical sensitivity of the paw (von Frey) in mice after 2 intramuscular injections of saline (20 µL; pH 4, pH 5, pH 6, pH 7.2) in a fatigue and a control group. To induce fatigue, mice were run for 2 h/day for 2 days prior to the first injection and 2 h/day for 2 days prior to the second injection. Muscle lactate, pCO 2 , pO 2 , creatinine kinase, phosphate, and histology were examined after the fatigue task and compared to a control group. Grip force was significantly decreased after 2 h of running indicating fatigue. The fatigue task did not induce muscle damage as there was no difference in muscle lactate, pCO 2 , pO 2 , creatinine kinase, phosphate, or histology. The fatigue task altered the dose-response relationship to intramuscular acidic saline injections. Mechanical hyperalgesia was observed in both fatigue and control groups after intramuscular injection of pH 4.0, but only the fatigue group after injection of pH 5. Neither the fatigue nor the control group developed hyperalgesia in response to intramuscular injection of pH 6 or pH 7.2. In conclusion, fatigue modified the susceptibility of mice to acid injection of pH 5.0 to result in mechanical hyperalgesia after 2 injections of pH 5.0. The fatigue task did not produce measurable changes in the muscle tissue suggesting a central mechanism mediating the enhancement of hyperalgesia.Perspective-These data therefore show that muscle fatigue can enhance the likelihood that one develops pain to a mild insult. Clinically, this could relate to the development of pain from such conditions as repetitive strain injury, and may relate to the interrelationship between chronic pain and fatigue. KeywordsFatigue; exercise; acid; protons; low-pH saline Pain arising from musculoskeletal disorders is a major clinical problem globally, 58 and fibromyalgia affects 2% of population in the United States. 57 As high as 76% of people with chronic widespread musculoskeletal pain conditions, such as fibromyalgia and arthritis, also report fatigue. 26 Conversely, as high as 94% of people with chronic fatigue syndrome report musculoskeletal pain and approximately half of the population has chronic widespread musculoskeletal pain. 56,60 Pain is increased during and after exercise in people with fibromyalgia and chronic fatigue syndrome. 20,48,54,55 Similarly, muscle fatigue is increased in people with chronic fatigue syndrome. 3,10,23 To assess mechanisms of chronic widespread pain, we developed an animal model that mimics the clinical symptoms. Two injections of acidic saline (pH 4.0), 5 days apart, into 1 muscle results in bilateral long-lasting hyperalgesia of the paw, 41 muscle,59 and the viscera28 without muscle tissue damage.41 The intramuscular pH decreases to a p...

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“…De leur côté, des mutations récessives du gène prx, codant pour la périaxine, ont été associées chez l'homme à deux types de neuropathies démyélinisantes héréditaires, une forme 4F de CMT et le syndrome récessif de Dejerine-Sottas (DSS) [18,19]. Par ailleurs, les souris dont le gène codant pour le dystroglycane dans les cellules de Schwann a été invalidé montrent une désorganisation importante des microvillosités au niveau des noeuds de Ranvier, une diminution de la densité de canaux Na + et un repliement de la myéline [20]. Le dystroglycane-α semble égale-ment être le récepteur de Mycobacterium leprae, un pathogène intracellulaire des cellules de Schwann qui induit une neuropathie périphérique [21].…”

Section: Connexion De La Cellule De Schwann à Sa Lame Basaleunclassified

Contacts cellulaires des fibres myélinisées du système nerveux périphérique

Oguievetskaia

Goutebroze

2006

J. Soc. Biol.

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Unique Role of Dystroglycan in Peripheral Nerve Myelination, Nodal Structure, and Sodium Channel Stabilization

Cited by 223 publications

References 61 publications

Mechanisms of Disease: congenital muscular dystrophies—glycosylation takes center stage

Mechanisms of Disease: congenital muscular dystrophies—glycosylation takes center stage

Muscle Fatigue Increases the Probability of Developing Hyperalgesia in Mice

Contacts cellulaires des fibres myélinisées du système nerveux périphérique

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