A mouse model of familial amyotrophic lateral sclerosis expressing a mutant superoxide dismutase 1 shows evidence of disordered transport in the vasopressin hypothalamo‐neurohypophysial axis

Aguilar, Jose Luis Gonzalez de; Gordon, Jon W.; Frydman, René; Lutz-Bucher, B.; Kienlen‐Campard, Pascal; Loeffler, Juergen

doi:10.1046/j.1460-9568.1999.00840.x

Cited by 21 publications

(8 citation statements)

References 48 publications

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“… 56 , 57 A large body of evidence shows that the AVP system is impaired in several neuromuscular diseases, such as amyotrophic lateral sclerosis and multiple sclerosis. 58 , 59 These data strengthen the hypothesis that AVP plays a physiological role in skeletal muscle homeostasis.…”

Section: Physiological Role Of Neurohypophyseal Hormones On Muscle Desupporting

confidence: 80%

Neurohypophyseal hormones: novel actors of striated muscle development and homeostasis

Costa

Rossi²,

Scicchitano

et al. 2014

Eur J Transl Myol

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Since the 1980’s, novel functional roles of the neurohypophyseal hormones vasopressin and oxytocin have emerged. Several studies have investigated the effects of these two neurohormones on striated muscle tissues, both in vitro and in vivo. The effects of vasopressin on skeletal myogenic cells, developing muscle and muscle homeostasis have been documented. Oxytocin appears to have a greater influence on cardiomyocite differentiation and heart homeostasis. This review summarizes the studies on these novel roles of the two neurohypophyseal hormones, and open the possibility of new therapeutic approaches for diseases affecting striated muscle.

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Section: Physiological Role Of Neurohypophyseal Hormones On Muscle Desupporting

confidence: 80%

Neurohypophyseal hormones: novel actors of striated muscle development and homeostasis

Costa

Rossi²,

Scicchitano

et al. 2014

Eur J Transl Myol

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“…Multiple independent reports document changes in AT in animal models of familial ALS (Borchelt et al, 1998; Bosco et al, 2010; Breuer and Atkinson, 1988; Collard et al, 1995; De Vos et al, 2007; Gonzalez de Aguilar et al, 1999; Marinkovic et al, 2012; Morfini et al, 2013; Morotz et al, 2012; Sasaki and Iwata, 1996; Sasaki et al, 2005). In contrast to AD, pathogenesis in ALS has been linked to activation of P38 MAP kinases (Ackerley et al, 2004; Bendotti et al, 2004; Bendotti et al, 2005).…”

Section: Introductionmentioning

confidence: 99%

Regulation of motor proteins, axonal transport deficits and adult-onset neurodegenerative diseases

Brady

Morfini

2017

Neurobiology of Disease

123

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Neurons affected in a wide variety of unrelated adult-onset neurodegenerative diseases (AONDs) typically exhibit a “dying back” pattern of degeneration, which is characterized by early deficits in synaptic function and neuritic pathology long before neuronal cell death. Consistent with this observation, multiple unrelated AONDs including Alzheimer’s disease, Parkinson’s disease, Huntington’s disease, and several motor neuron diseases feature early alterations in kinase-based signaling pathways associated with deficits in axonal transport (AT), a complex cellular process involving multiple intracellular trafficking events powered by microtubule-based motor proteins. These pathogenic events have important therapeutic implications, suggesting that a focus on preservation of neuronal connections may be more effective to treat AONDs than addressing neuronal cell death. While the molecular mechanisms underlying AT abnormalities in AONDs are still being analyzed, evidence has accumulated linking those to a well-established pathological hallmark of multiple AONDs: altered patterns of neuronal protein phosphorylation. Here, we present a short overview on the biochemical heterogeneity of major motor proteins for AT, their regulation by protein kinases, and evidence revealing cell type-specific AT specializations. When considered together, these findings may help explain how independent pathogenic pathways can affect AT differentially in the context of each AOND.

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“…Somewhat unique among extra-motor features in ALS are alterations in energy metabolism [ 7 ], autonomic function [ 8 ], and in some patients [ 9 , 10 ] and animal models [ 11 ], involvement of the hypothalamic-pituitary axis. Deficits in energy metabolism are well recognized in ALS, with a paradoxical hypermetabolism occurring in many patients that combines with dysphagia to result in malnourishment and loss of body mass index (BMI) [ 12 ].…”

Section: Introductionmentioning

confidence: 99%

TDP-43 pathology in the basal forebrain and hypothalamus of patients with amyotrophic lateral sclerosis

Cykowski

Takei

Schulz

et al. 2014

acta neuropathol commun

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IntroductionAmyotrophic lateral sclerosis is a neurodegenerative disease characterized clinically by motor symptoms including limb weakness, dysarthria, dysphagia, and respiratory compromise, and pathologically by inclusions of transactive response DNA-binding protein 43 kDa (TDP-43). Patients with amyotrophic lateral sclerosis also may demonstrate non-motor symptoms and signs of autonomic and energy dysfunction as hypermetabolism and weight loss that suggest the possibility of pathology in the forebrain, including hypothalamus. However, this region has received little investigation in amyotrophic lateral sclerosis. In this study, the frequency, topography, and clinical associations of TDP-43 inclusion pathology in the basal forebrain and hypothalamus were examined in 33 patients with amyotrophic lateral sclerosis: 25 men and 8 women; mean age at death of 62.7 years, median disease duration of 3.1 years (range of 1.3 to 9.8 years).ResultsTDP-43 pathology was present in 11 patients (33.3%), including components in both basal forebrain (n= 10) and hypothalamus (n= 7). This pathology was associated with non-motor system TDP-43 pathology (Χ2= 17.5, p= 0.00003) and bulbar symptoms at onset (Χ2= 4.04, p= 0.044), but not age or disease duration. Furthermore, TDP-43 pathology in the lateral hypothalamic area was associated with reduced body mass index (W= 11, p= 0.023).ConclusionsThis is the first systematic demonstration of pathologic involvement of the basal forebrain and hypothalamus in amyotrophic lateral sclerosis. Furthermore, the findings suggest that involvement of the basal forebrain and hypothalamus has significant phenotypic associations in amyotrophic lateral sclerosis, including site of symptom onset, as well as deficits in energy metabolism with loss of body mass index.

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A mouse model of familial amyotrophic lateral sclerosis expressing a mutant superoxide dismutase 1 shows evidence of disordered transport in the vasopressin hypothalamo‐neurohypophysial axis

Cited by 21 publications

References 48 publications

Neurohypophyseal hormones: novel actors of striated muscle development and homeostasis

Neurohypophyseal hormones: novel actors of striated muscle development and homeostasis

Regulation of motor proteins, axonal transport deficits and adult-onset neurodegenerative diseases

TDP-43 pathology in the basal forebrain and hypothalamus of patients with amyotrophic lateral sclerosis

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