A panel of macroautophagy markers in lymphomonocytes of patients with amyotrophic lateral sclerosis

Sala, Gessica; Tremolizzo, Lucio; Melchionda, Laura; Stefanoni, Giovanni; Derosa, Matteo; Susani, Emanuela; Pagani, Ambrogio; Perini, Michele; Pettini, P; Tavernelli, F; Zarcone, Davide; Ferrarese, Carlo

doi:10.3109/17482968.2011.611139

Cited by 8 publications

(3 citation statements)

References 22 publications

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“…It is interesting to note that in Parkinson's disease (PD) and in amyotrophic lateral sclerosis (ALS), autophagy status in PBMCs was also studied. PBMCs from PD patients displayed a reduced expression of the chaperone-mediated autophagy carrier hsc70 protein [ 68 ] and results from PBMCs from ALS patients are contradictory: No significant difference of Beclin-1, LC3 immunoreactivity and mRNA Atg12 expression was observed between patients and controls although PBMC displayed a clear macroautophagy induction following exposure to rapamycin and lithium [ 69 ], while other data showed that the expression of the early autophagy-associated gene ATG12 was increased in PBMC from SCA7 patients in correlation with disease severity [ 70 ].…”

Section: Discussionmentioning

confidence: 99%

Inflammatory Stress on Autophagy in Peripheral Blood Mononuclear Cells from Patients with Alzheimer's Disease during 24 Months of Follow-Up

et al. 2015

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Recent findings indicate that microglia in Alzheimer’s disease (AD) is senescent whereas peripheral blood mononuclear cells (PBMCs) could infiltrate the brain to phagocyte amyloid deposits. However, the molecular mechanisms involved in the amyloid peptide clearance remain unknown. Autophagy is a physiological degradation of proteins and organelles and can be controlled by pro-inflammatory cytokines. The purpose of this study was to evaluate the impact of inflammation on autophagy in PBMCs from AD patients at baseline, 12 and 24 months of follow-up. Furthermore, PBMCs from healthy patients were also included and treated with 20 μM amyloid peptide 1–42 to mimic AD environment. For each patient, PBMCs were stimulated with the mitogenic factor, phytohaemagglutin (PHA), and treated with either 1 μM C16 as an anti-inflammatory drug or its vehicle. Autophagic markers (Beclin-1, p62/sequestosome 1 and microtubule-associated protein-light chain 3: LC3) were quantified by western blot and cytokines (Interleukin (IL)-1β, Tumor necrosis Factor (TNF)-α and IL-6) by Luminex X-MAP® technology. Beclin-1 and TNF-α levels were inversely correlated in AD PBMCs at 12 months post-inclusion. In addition, Beclin-1 and p62 increased in the low inflammatory environment induced by C16. Only LC3-I levels were inversely correlated with cognitive decline at baseline. For the first time, this study describes longitudinal changes in autophagic markers in PBMCs of AD patients under an inflammatory environment. Inflammation would induce autophagy in the PBMCs of AD patients while an anti-inflammatory environment could inhibit their autophagic response. However, this positive response could be altered in a highly aggressive environment.

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Section: Discussionmentioning

confidence: 99%

Inflammatory Stress on Autophagy in Peripheral Blood Mononuclear Cells from Patients with Alzheimer's Disease during 24 Months of Follow-Up

et al. 2015

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“…Peripheral blood mononuclear cells (PBMC) isolation was obtained by Ficoll-Histopaque density gradient centrifugation as described before [18]. PBMC pellets were then stocked at −80°C until assessment.…”

Section: Methodsmentioning

confidence: 99%

Valproate Treatment in an ALS Patient Carrying a c.194G>A Spastin Mutation and SMN2 Homozygous Deletion

Tremolizzo

Sala

Conti

et al. 2014

Case Reports in Neurological Medicine

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Here we report the case of an ALS patient found to carry both a novel heterozygous change (c.194G>A) within the spastin gene and a homozygous deletion of the SMN2 gene. The patient was started on valproic acid (VPA, 600 mg/die per os) considering the capacity of this drug of increasing survival motor neuron through an epigenetic mechanism. Patient clinical course and molecular effects of VPA on skin fibroblasts obtained from the proband are described. This c.194G>A spastin mutation might expand the previously known borders of type 4 spastic paraplegia (SPG4) and we suggest the intriguing possibility that the absence of SMN2 might have acted as a contributory risk factor for starting lower motor neuron damage. Exploring the relationship genocopy-phenocopy in selected ALS patients might represent an interesting strategy for understanding its clinical variability.

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“…The pathology of ALS/FTD is typically accompanied by marked alterations in a key physiological process that is related to energy homeostasis: autophagy ( Sala et al, 2012 ). Since the deletion of autophagy-related genes is sufficient to cause neuronal death, autophagy is considered an essential homeostatic process for neurons ( Evans and Holzbaur, 2019 ).…”

Section: Defects Of Nutrient Signaling and Autophagy In Als/ftdmentioning

confidence: 99%

Defects of Nutrient Signaling and Autophagy in Neurodegeneration

Ondaro

Hernandez-Eguiazu

Garciandia-Arcelus

et al. 2022

Front. Cell Dev. Biol.

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A panel of macroautophagy markers in lymphomonocytes of patients with amyotrophic lateral sclerosis

Cited by 8 publications

References 22 publications

Inflammatory Stress on Autophagy in Peripheral Blood Mononuclear Cells from Patients with Alzheimer's Disease during 24 Months of Follow-Up

Inflammatory Stress on Autophagy in Peripheral Blood Mononuclear Cells from Patients with Alzheimer's Disease during 24 Months of Follow-Up

Valproate Treatment in an ALS Patient Carrying a c.194G>A Spastin Mutation and SMN2 Homozygous Deletion

Defects of Nutrient Signaling and Autophagy in Neurodegeneration

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