LIVIVO - Search results -

Search results

Result 1 - 10 of total 111

Search options

Article ; Online: Prionoids in amyotrophic lateral sclerosis.

Gosset, Philippe / Camu, William / Raoul, Cedric / Mezghrani, Alexandre

Brain communications

2022 Volume 4, Issue 3, Page(s) fcac145

Abstract: Amyotrophic lateral sclerosis (ALS) is the third most frequent neurodegenerative disease after Alzheimer's and Parkinson's disease. ALS is characterized by the selective and progressive loss of motoneurons in the spinal cord, brainstem and cerebral ... ...

Abstract	Amyotrophic lateral sclerosis (ALS) is the third most frequent neurodegenerative disease after Alzheimer's and Parkinson's disease. ALS is characterized by the selective and progressive loss of motoneurons in the spinal cord, brainstem and cerebral cortex. Clinical manifestations typically occur in midlife and start with focal muscle weakness, followed by the rapid and progressive wasting of muscles and subsequent paralysis. As with other neurodegenerative diseases, the condition typically begins at an initial point and then spreads along neuroanatomical tracts. This feature of disease progression suggests the spreading of prion-like proteins called prionoids in the affected tissues, which is similar to the spread of prion observed in Creutzfeldt-Jakob disease. Intensive research over the last decade has proposed the ALS-causing gene products Cu/Zn superoxide dismutase 1, TAR DNA-binding protein of 43 kDa, and fused in sarcoma as very plausible prionoids contributing to the spread of the pathology. In this review, we will discuss the molecular and cellular mechanisms leading to the propagation of these prionoids in ALS.
Language	English
Publishing date	2022-06-09
Publishing country	England
Document type	Journal Article ; Review
ISSN	2632-1297
ISSN (online)	2632-1297
DOI	10.1093/braincomms/fcac145
Database	MEDical Literature Analysis and Retrieval System OnLINE

Order via subito

This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

Inter-library loan at ZB MED

Your chosen title can be delivered directly to ZB MED Cologne location if you are registered as a user at ZB MED Cologne.

Article: Editorial: Vitamin D in Neurological Diseases: From Pathophysiology to Therapy.

Smolders, Joost / Hiller, Amie / Camu, William

Frontiers in neurology

2021 Volume 12, Page(s) 614900

Language	English
Publishing date	2021-03-09
Publishing country	Switzerland
Document type	Editorial
ZDB-ID	2564214-5
ISSN	1664-2295
ISSN	1664-2295
DOI	10.3389/fneur.2021.614900
Database	MEDical Literature Analysis and Retrieval System OnLINE

Order via subito

Article ; Online: Teriflunomide-induced psoriasiform changes of fingernails: a new example of paradoxical side effect?

Dereure, Olivier / Camu, William

International journal of dermatology

2017 Volume 56, Issue 12, Page(s) 1479–1481

MeSH term(s)	Adult ; Crotonates/adverse effects ; Drug Eruptions/etiology ; Female ; Humans ; Immunologic Factors/adverse effects ; Multiple Sclerosis/drug therapy ; Nail Diseases/chemically induced ; Psoriasis/chemically induced ; Toluidines/adverse effects
Chemical Substances	Crotonates ; Immunologic Factors ; Toluidines ; teriflunomide (1C058IKG3B)
Language	English
Publishing date	2017-09-07
Publishing country	England
Document type	Case Reports ; Letter
ZDB-ID	412254-9
ISSN	1365-4632 ; 0011-9059 ; 1461-1244
ISSN (online)	1365-4632
ISSN	0011-9059 ; 1461-1244
DOI	10.1111/ijd.13742
Database	MEDical Literature Analysis and Retrieval System OnLINE

Full text online

Accessible to users with ZB MED library card

In stock of ZB MED Cologne/Königswinter

Ui VI Zs.189: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾

Article ; Online: An Update on Vitamin D and Disease Activity in Multiple Sclerosis.

Smolders, Joost / Torkildsen, Øivind / Camu, William / Holmøy, Trygve

CNS drugs

2019 Volume 33, Issue 12, Page(s) 1187–1199

Abstract: Vitamin D and its main active metabolite 1,25-dihydroxyvitamin D serve a crucial role in maintenance of a healthy calcium metabolism, yet have additional roles in immune and central nervous system cell homeostasis. Serum levels of 25-hydroxyvitamin D are ...

Abstract	Vitamin D and its main active metabolite 1,25-dihydroxyvitamin D serve a crucial role in maintenance of a healthy calcium metabolism, yet have additional roles in immune and central nervous system cell homeostasis. Serum levels of 25-hydroxyvitamin D are a biomarker of future disease activity in patients with early relapsing-remitting multiple sclerosis (RRMS), and vitamin D supplementation in patients with low circulating 25-dihydroxyvitamin D levels has been anticipated as a potential efficacious treatment strategy. The results of the first large randomized clinical trials (RCTs), the SOLAR and CHOLINE studies, have now been published. The SOLAR study compared 14,000 IU of vitamin D
MeSH term(s)	Animals ; Dietary Supplements ; Disease Progression ; Humans ; Interferon beta-1a/metabolism ; Multiple Sclerosis/metabolism ; Observational Studies as Topic ; Randomized Controlled Trials as Topic ; Vitamin D/analogs & derivatives ; Vitamin D/metabolism
Chemical Substances	Vitamin D (1406-16-2) ; 25-hydroxyvitamin D (A288AR3C9H) ; Interferon beta-1a (XRO4566Q4R)
Language	English
Publishing date	2019-11-04
Publishing country	New Zealand
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Review
ZDB-ID	1203800-3
ISSN	1179-1934 ; 1172-7047
ISSN (online)	1179-1934
ISSN	1172-7047
DOI	10.1007/s40263-019-00674-8
Database	MEDical Literature Analysis and Retrieval System OnLINE

Full text online

Accessible to users with ZB MED library card

In stock of ZB MED Cologne/Königswinter

Zs.A 4083: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
Jg. 1995 - 2021: Lesesall (2.OG)
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾

Article ; Online: The

Ceprian, Maria / Juntas-Morales, Raul / Campbell, Graham / Walther-Louvier, Ulrike / Rivier, François / Camu, William / Esselin, Florence / Echaniz-Laguna, Andoni / Stojkovic, Tanya / Bouhour, Françoise / Latour, Philippe / Tricaud, Nicolas

International journal of molecular sciences

2024 Volume 25, Issue 8

Abstract: Demyelinating Charcot-Marie-Tooth 4G (CMT4G) results from a recessive mutation in the 5'UTR region of the Hexokinase 1 (HK1) gene. HK participates in mitochondrial calcium homeostasis by binding to the Voltage-Dependent Anion Channel (VDAC), through its ... ...

Abstract	Demyelinating Charcot-Marie-Tooth 4G (CMT4G) results from a recessive mutation in the 5'UTR region of the Hexokinase 1 (HK1) gene. HK participates in mitochondrial calcium homeostasis by binding to the Voltage-Dependent Anion Channel (VDAC), through its N-terminal porin-binding domain. Our hypothesis is that CMT4G mutation results in a broken interaction between mutant HK1 and VDAC, disturbing mitochondrial calcium homeostasis. We studied a cohort of 25 CMT4G patients recruited in the French gypsy population. The disease was characterized by a childhood onset, an intermediate demyelinating pattern, and a significant phenotype leading to becoming wheelchair-bound by the fifth decade of life. Co-IP and PLA studies indicated a strong decreased interaction between VDAC and HK1 in the patients' PBMCs and sural nerve. We observed that either wild-type HK1 expression or a peptide comprising the 15 aa of the N-terminal wild-type HK1 administration decreased mitochondrial calcium release in HEK293 cells. However, mutated CMT4G HK1 or the 15 aa of the mutated HK1 was unable to block mitochondrial calcium release. Taken together, these data show that the CMT4G-induced modification of the HK1 N-terminus disrupts HK1-VDAC interaction. This alters mitochondrial calcium buffering that has been shown to be critical for myelin sheath maintenance.
MeSH term(s)	Humans ; Hexokinase/genetics ; Hexokinase/metabolism ; Charcot-Marie-Tooth Disease/genetics ; Charcot-Marie-Tooth Disease/metabolism ; Calcium/metabolism ; Mitochondria/metabolism ; Mitochondria/genetics ; Mutation ; Female ; HEK293 Cells ; Male ; 5' Untranslated Regions/genetics ; Voltage-Dependent Anion Channel 1/metabolism ; Voltage-Dependent Anion Channel 1/genetics ; Adult ; Protein Binding ; Adolescent ; Middle Aged ; Child ; Young Adult
Chemical Substances	Hexokinase (EC 2.7.1.1) ; Calcium (SY7Q814VUP) ; HK1 protein, human (EC 2.7.1.1) ; 5' Untranslated Regions ; Voltage-Dependent Anion Channel 1 (EC 1.6.-)
Language	English
Publishing date	2024-04-15
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2019364-6
ISSN	1422-0067 ; 1422-0067 ; 1661-6596
ISSN (online)	1422-0067
ISSN	1422-0067 ; 1661-6596
DOI	10.3390/ijms25084364
Database	MEDical Literature Analysis and Retrieval System OnLINE

Order via subito

Article: Epidémiologie et traitement de la sclérose latérale amyotrophique.

Camu, William

Soins; la revue de reference infirmiere

2008 , Issue 726, Page(s) 40–43

Title translation	Epidemiology and treatment of amyotrophic lateral sclerosis.
MeSH term(s)	Amyotrophic Lateral Sclerosis/diagnosis ; Amyotrophic Lateral Sclerosis/epidemiology ; Amyotrophic Lateral Sclerosis/etiology ; Amyotrophic Lateral Sclerosis/therapy ; Causality ; Diagnosis, Differential ; Excitatory Amino Acid Antagonists/therapeutic use ; France/epidemiology ; Humans ; Incidence ; Palliative Care/organization & administration ; Patient Care Team/organization & administration ; Prevalence ; Prognosis ; Riluzole/therapeutic use
Chemical Substances	Excitatory Amino Acid Antagonists ; Riluzole (7LJ087RS6F)
Language	French
Publishing date	2008-06
Publishing country	France
Document type	Journal Article
ZDB-ID	604655-1
ISSN	0038-0814
ISSN	0038-0814
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Zs.B 2545: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾
- See ZB MED holdings
- Order with fees

Article ; Online: Repeated neurofilament light chain measurements did not capture Riluzole therapeutic effect in amyotrophic lateral sclerosis patients.

Esselin, Florence / De la Cruz, Elisa / Hirtz, Christophe / Tiers, Laurent / Alphandery, Sébastien / Baudesson, Léandra / Taieb, Guillaume / Camu, William / Lehmann, Sylvain

CNS neuroscience & therapeutics

2022 Volume 28, Issue 10, Page(s) 1532–1538

Abstract: Background: Little is known about the influence of Riluzole on serum neurofilament light chain (sNfL) levels, a biomarker of prognosis in amyotrophic lateral sclerosis (ALS), and variations with time of sNfL concentrations are controversial.: Methods!# ...

Abstract	Background: Little is known about the influence of Riluzole on serum neurofilament light chain (sNfL) levels, a biomarker of prognosis in amyotrophic lateral sclerosis (ALS), and variations with time of sNfL concentrations are controversial. Methods: Sera from ALS patients (n = 141) and controls (n = 33) were collected at inclusion (sNfL1) and second visit (sNfL2, mean delay 10.4 ± 8.7 months). sNfL levels, determined by single-molecule array, were compared between ALS and controls at both time points. sNfL concentration changes were compared between patients with Riluzole (w/Ril) at inclusion in the study and those who were treated by Riluzole following inclusion (w/o Ril). The factors influencing sNfL concentrations and changes were studied using linear regression and multivariate analysis. Results: sNfL levels were higher in ALS patients than in controls at the two time points (p < 0.00001). In ALS patients, sNfL concentrations were higher in females for both sNfL1 (p = 0.014) and sNfL2 (p < 0.001). In the whole ALS group, sNfL levels were higher at sNfL2 than at sNfL1 (p < 0.001). sNfL1 and sNfL2 concentrations were similar between the two ALS subgroups (w/ and w/o Ril). ALS functional rating scale-revised rate of decline and gender were the two main factors significantly influencing both sNfL1 and sNfL2 levels (p < 0.01). However, only gender was shown to significantly influence sNfL changes with time (p = 0.003). Conclusions: In this study, sNfL levels increased with time in ALS patients and there was no difference between subjects already treated by Riluzole and those treated after sNfL1. Further studies with larger population samples and different sampling intervals are warranted to better determine the real potential of sNfL measurement as a tool to monitor treatment response in ALS.
MeSH term(s)	Amyotrophic Lateral Sclerosis/diagnosis ; Amyotrophic Lateral Sclerosis/drug therapy ; Biomarkers ; Female ; Humans ; Intermediate Filaments ; Neurofilament Proteins ; Prognosis ; Riluzole/therapeutic use
Chemical Substances	Biomarkers ; Neurofilament Proteins ; Riluzole (7LJ087RS6F)
Language	English
Publishing date	2022-06-25
Publishing country	England
Document type	Journal Article
ZDB-ID	2423461-8
ISSN	1755-5949 ; 1755-5930
ISSN (online)	1755-5949
ISSN	1755-5930
DOI	10.1111/cns.13894
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Zs.A 4537: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
Jg. 1995 - 2021: Lesesall (2.OG)
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾
- See ZB MED holdings
- Order with fees

Article ; Online: Ultrasensitive digital immunoassays for SOD1 conformation in amyotrophic lateral sclerosis.

Morichon, Lisa / Hirtz, Christophe / Tiers, Laurent / Mezghrani, Alexandre / Raoul, Cédric / Esselin, Florence / La Cruz, Elisa De / Julien, Jean-Pierre / Camu, William / Lehmann, Sylvain

Bioanalysis

2023 Volume 15, Issue 15, Page(s) 927–936

Abstract: Aim: ...

Abstract	Aim:
MeSH term(s)	Humans ; Amyotrophic Lateral Sclerosis ; Superoxide Dismutase-1 ; Biological Assay ; Immunoassay ; Molecular Conformation
Chemical Substances	Superoxide Dismutase-1 (EC 1.15.1.1) ; SOD1 protein, human
Language	English
Publishing date	2023-08-31
Publishing country	England
Document type	Journal Article
ISSN	1757-6199
ISSN (online)	1757-6199
DOI	10.4155/bio-2023-0103
Database	MEDical Literature Analysis and Retrieval System OnLINE

Order via subito

Inter-library loan at ZB MED

Your chosen title can be delivered directly to ZB MED Cologne location if you are registered as a user at ZB MED Cologne.

Article: Early-onset, conjugal, twin-discordant, and clusters of sporadic ALS: Pathway to discovery of etiology

Spencer, Peter S / Palmer, Valerie S / Kisby, Glen E / Lagrange, Emmeline / Horowitz, B Zane / Valdes Angues, Raquel / Reis, Jacques / Vernoux, Jean-Paul / Raoul, Cédric / Camu, William

Frontiers in neuroscience

2023 Volume 17, Page(s) 1005096

Abstract: The identity and role of environmental factors in the etiology of sporadic amyotrophic lateral sclerosis (sALS) is poorly understood outside of three former high-incidence foci of Western Pacific ALS and a hotspot of sALS in the French Alps. In both ... ...

Abstract	The identity and role of environmental factors in the etiology of sporadic amyotrophic lateral sclerosis (sALS) is poorly understood outside of three former high-incidence foci of Western Pacific ALS and a hotspot of sALS in the French Alps. In both instances, there is a strong association with exposure to DNA-damaging (genotoxic) chemicals years or decades prior to clinical onset of motor neuron disease. In light of this recent understanding, we discuss published geographic clusters of ALS, conjugal cases, single-affected twins, and young-onset cases in relation to their demographic, geographic and environmental associations but also whether, in theory, there was the possibility of exposure to genotoxic chemicals of natural or synthetic origin. Special opportunities to test for such exposures in sALS exist in southeast France, northwest Italy, Finland, the U.S. East North Central States, and in the U.S. Air Force and Space Force. Given the degree and timing of exposure to an environmental trigger of ALS may be related to the age at which the disease is expressed, research should focus on the lifetime exposome (from conception to clinical onset) of young sALS cases. Multidisciplinary research of this type may lead to the identification of ALS causation, mechanism, and primary prevention, as well as to early detection of impending ALS and pre-clinical treatment to slow development of this fatal neurological disease.
Language	English
Publishing date	2023-02-13
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	2411902-7
ISSN	1662-453X ; 1662-4548
ISSN (online)	1662-453X
ISSN	1662-4548
DOI	10.3389/fnins.2023.1005096
Database	MEDical Literature Analysis and Retrieval System OnLINE

Order via subito

Article ; Online: Serum neurofilament light chain cut-off definition for clinical diagnosis and prognosis of amyotrophic lateral sclerosis.

Brousse, Mehdi / Delaby, Constance / De La Cruz, Elisa / Kuhle, Jens / Benkert, Pascal / Mondesert, Etienne / Ginestet, Nelly / Hirtz, Christophe / Camu, William / Lehmann, Sylvain / Esselin, Florence

European journal of neurology

2023 Volume 30, Issue 7, Page(s) 1919–1927

Abstract: Background: The neurofilament light chain (NfL) assay is gradually becoming an essential diagnostic tool for the diagnosis of many neurological diseases including amyotrophic lateral sclerosis (ALS). Different methods for the determination of this ... ...

Abstract	Background: The neurofilament light chain (NfL) assay is gradually becoming an essential diagnostic tool for the diagnosis of many neurological diseases including amyotrophic lateral sclerosis (ALS). Different methods for the determination of this biomarker in serum have been developed in recent years. Methods: We measured blood NfL in 429 patients referred to the tertiary ALS center of Montpellier, France using two different ultrasensitive methods (Ella™ and Simoa™) and we compared the clinical performances of these two approaches. We also converted NfL values into age and body mass index-adjusted Z-scores to assess cut-off values of this biomarker in this clinical context. Results: We show comparable diagnostic and prognostic performance of Ella™ and Simoa™ technologies in ALS, with specificities and sensitivities exceeding 80% for both. We propose cut-off values for serum NfL in this clinical context, thus enabling the routine clinical use of this biomarker. Conclusion: The use of NfL in routine clinical practice will help predict survival and improve diagnostic accuracy by distinguishing ALS from other neurological diseases and motor neuron disease mimics.
MeSH term(s)	Humans ; Amyotrophic Lateral Sclerosis/diagnosis ; Intermediate Filaments ; Prognosis ; Biomarkers ; Neurofilament Proteins ; Body Mass Index
Chemical Substances	Biomarkers ; Neurofilament Proteins
Language	English
Publishing date	2023-04-19
Publishing country	England
Document type	Journal Article
ZDB-ID	1280785-0
ISSN	1468-1331 ; 1351-5101 ; 1471-0552
ISSN (online)	1468-1331
ISSN	1351-5101 ; 1471-0552
DOI	10.1111/ene.15813
Database	MEDical Literature Analysis and Retrieval System OnLINE

Full text online

Accessible to users with ZB MED library card

In stock of ZB MED Cologne/Königswinter

Zs.A 4738: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (2.OG) ab Jg. 2022: Lesesaal (EG)
Zs.MO 592: Show issues

Order via subito

Details ▾

To top

More links

Kategorien

Order via subito

Inter-library loan at ZB MED

More links

Kategorien

Order via subito

Full text online

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

Full text online

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

More links

Kategorien

Order via subito

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

More links

Kategorien

Order via subito

Inter-library loan at ZB MED

More links

Kategorien

Order via subito

Full text online

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito