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Mechanisms and treatment strategies of demyelinating and dysmyelinating Charcot-Marie-Tooth disease

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Neural Regeneration Research 2023年第9期18卷 1931-1939页

作者： Nadège Hertzog Claire Jacob Faculty of Biology Institute of Developmental Biology and NeurobiologyJohannes Gutenberg University MainzHanns-Dieter-Hüsch-WegMainzGermany

Schwann cells,the myelinating glia of the peripheral nervous system,wrap axons multiple times to build their myelin *** is of paramount importance for axonal integrity and fast axon potential ***,myelin is lacking or dysfunctional in several neuropathies including demyelinating and dysmyelinating Charcot-M arie-To oth ***-Marie-To oth disease represents the most prevalent inherited neuropathy in humans and is classified either as axonal,demyelinating or dysmyelinating,or as *** demyelinating or dysmyelinating forms of Charcot-Marie-Tooth disease constitute the majority of the disease cases and are most frequently due to mutations in the three following myelin genes:peripheral myelin protein 22,myelin protein ze ro and gap junction beta 1(coding for Connexin 32) causing Charcot-M arie-Tooth disease type 1A,Charcot-Marie-Tooth disease type 1B,and X-linked Charcot-M arie-Tooth disease type 1,*** resulting perturbation of myelin structure and function leads to axonal demyelination or dysmyelination and causes severe disabilities in affected *** treatment to cure or slow down the disease progression is currently available on the market,howeve r,scientific discoveries led to a better understanding of the pathomechanisms of the disease and to potential treatment *** this review,we describe the features and molecular mechanisms of the three main demyelinating or dysmyelinating forms of Charcot-Marie-Tooth disease,the rodent models used in research,and the emerging therapeutic approaches to cure or counteract the progression of the disease.

关键词： Charcot-Marie-Tooth disease rodent models emerging treatments demyelination and dysmyelination endoplasmic reticulum stress gene therapy myelin repair Schwann cells unfolded protein response

Phase separating Rho:a widespread regulatory function of disordered regions in proteins revealed in bacteria

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Signal Transduction and Targeted Therapy 2023年第7期8卷 3203-3205页

作者： Eric Schumbera Pablo Mier Miguel A.Andrade-Navarro Institute of Organismic and Molecular Evolution Faculty of BiologyJohannes Gutenberg University of Mainz55128 MainzGermany

A recent study published in Science by Krypotou et *** the role of an intrinsically disordered region(IDR)in the transcription termination Rho protein from Bacteroides thetaiotaomicron:in response to starvation Rho phase separated,which triggered an increase in Rho activity and expression changes.1 These results demonstrate that a highly conserved protein can acquire new regulatory properties in evolution by gaining an IDR that leads to protein phase separation.

关键词： al. disordered separating

Apolipoprotein A1,the neglected relative of Apolipoprotein E and its potential role in Alzheimer’s disease

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Neural Regeneration Research 2021年第11期16卷 2141-2148页

作者： Kristina Endres Department of Psychiatry and Psychotherapy University Medical Center of the Johannes Gutenberg-University MainzUntere Zahlbacher Str.855131 MainzGermany

Lipoproteins are multi-molecule assemblies with the primary function of transportation and processing of lipophilic substances within aqueous bodily fluids(blood,cerebrospinal fluid).Nevertheless,they also exert other physiological functions such as immune *** particular,neurons are both sensitive to uncontrolled responses of the immune system and highly dependent on a controlled and sufficient supply of *** this reason,the role of certain lipoproteins and their protein-component(apolipoproteins,Apo’s)in neurological diseases is ***,for example,is well-accepted as one of the major risk factors for sporadic Alzheimer’s disease with a protective allele variant(ε2)and a risk-causing allele variant(ε4).ApoA1,the major protein component of high-density lipoproteins,is responsible for transportation of excess cholesterol from peripheral tissues to the *** protein is synthesized in the liver and intestine but also can enter the brain via the choroid plexus and thereby might have an impact on brain lipid *** review focuses on the role of ApoA1 in Alzheimer’s disease and discusses whether its role within this neurodegenerative disorder is specific or represents a general neuroprotective mechanism.

关键词： Aβ ApoA1 cholesterol high-density lipoproteins lipids lipoprotein neurodegeneration senile plaque

Last line therapy with sorafenib in colorectal cancer: A retrospective analysis

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World Journal of Gastroenterology 2016年第23期22卷 5400-5405页

作者： Ksenia Martchenko Irene Schmidtmann Thomas Thomaidis Verena Thole Peter R Galle Marc Becker Markus Mohler Thomas C Wehler Carl C Schimanski Department of Internal Medicine Marienhospital 64285 Darmstadt Germany Institute of Medical Biostatistics Epidemiology and Informatics Johannes Gutenberg University Hospital of Mainz 55131 Mainz Germany First Department of Internal Medicine Johannes Gutenberg University Hospital of Mainz 55131 Mainz Germany Department of Internal Medicine Katholische Kliniken Mainz 55131 Mainz Germany Institute of Pathology Saarland University Medical Center 66421 Homburg Germany Third Department of Internal Medicine Johannes Gutenberg University Hospital of Mainz 55131 Mainz Germany

AIM: To analyze the efficacy of last line sorafenib treatment in colorectal cancer patients. METHODS: All patients receiving chemotherapy for colorectal cancer in the outpatient clinic of the university of mainz since 2006 were retrospectively analyzed for last line sorafenib exposure. Charts of identified patients were analyzed for clinic-pathological parameters, like data on gender, age, date of initial diagnosis, UICC stage, number and kind of the pretherapies, therapy start and end of sorafenib, sorafenib mediated treatment cessation, side effects, response rates, time to progression and overall survival. RESULTS: Ten patients with a median of 3.0 prior chemotherapy lines had received a last line sorafenib therapy either alone(10%) or in combination with 5-fluorouracil derivates(90%). All patients suffered from colorectal cancer stage UICC 4 and were routinely seen in 2-wk intervals in the oncology outpatient clinic. Median duration of treatment was 142.0 d. At 8 wk 80% of patients showed stable disease but we did not observe any remissions. Median time to progression was 140.5 d(4.7 mo), while median overall survival reached 176.5 d. One patient ceased treatment due to side effects. Reason for treatment stop was bleeding complication in one case and non-specified sorafenib intolerance in another case. Due to the retrospective approach we did not further quantify side ***: This retrospective analysis encourages further investigation of sorafenib in colorectal cancer last line therapy.

关键词： Colorectal cancer Sorafenib Regorafenib Chemotherapy Last line

Synchronized ventilation during resuscitation in pigs does not necessitate high inspiratory pressures to provide adequate oxygenation

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World Journal of Emergency Medicine 2023年第5期14卷 393-396页

作者： Miriam Renz Raphael RenéCinto Noack RenéRissel Katja Mohnke Julian Riedel Bastian Dunges Alexander Ziebart Erik Kristoff er Hartmann Robert Rummler Department of Anesthesiology University Medical CenterJohannes Gutenberg UniversityMainz 55131Germany

The optimal ventilation method for patients suffering from cardiac arrest and receiving cardiopulmonary resuscitation(CPR)remains unclear,and the recent guidelines do not provide detailed information.[1-3]During CPR,changing thoracic pressures due to chest compressions and ventilation pressure infl uence venous return and cardiac output,respectively.[4-6]Novel mechanical ventilation modes that are synchronized to chest compressions may improve blood flow and oxygenation.[4]In this porcine trial,we investigated the feasibility of a specially designed chest compression synchronized ventilation(CCSV)mode with peak pressures limited to 40 mbar(1 mbar=0.1 kPa)and an experimental synchronized ventilation(SV)limited to 20 mbar,respectively,intended to achieve a more lung-protective ventilation pattern during *** evaluated whether the diff erent synchronized pressure levels ameliorate hemodynamics,gas exchange and pulmonary function and compared it to intermittent positive pressure ventilation(IPPV).

关键词： resuscitation protective cardiac

Type 1 diabetes and associated autoimmune diseases

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World Journal of Diabetes 2020年第11期11卷 527-539页

作者： Lara Frommer George J Kahaly Department of Medicine I Johannes Gutenberg Medical CenterMainz 55131Germany

BACKGROUND Common autoimmune diseases(AID)tend to occur together in the same individual and *** 1 diabetes(T1D)is caused by an autoimmuneinduced inflammatory destruction of the pancreatic tissue and clusters with several other *** To compare the demographic,clinical,and serological features of patients with single T1D vs those with T1D and associated *** From October 1999 to February 2020,a total of 665 patients with T1D and their first-degree relatives were *** Compared to patients with isolated T1D,those with T1D+AID were older and had a higher female:male *** patient age and age at disease onset were higher in T1D+AID vs T1D *** average time interval between T1D onset and the onset of a second glandular AID was markedly shorter than the time interval between T1D and the occurrence of a non-endocrine AID.T1Dspecific autoantibodies were more frequent in patients with T1D+AID and relatives vs those with T1D ***,the prevalence of AID and autoantibodies against various tissues were found to be higher in relatives of patients with T1D only compared to relatives of patients with T1D+*** Annual serological and subsequent functional screening for AID in patients with T1D and their first-degree relatives is recommended.

关键词： Type 1 diabetes Autoimmunity Serology Antibodies Autoimmune endocrine diseases Autoimmune non-endocrine disorders

Spreading depression and focal venous cerebral ischemia enhance cortical neurogenesis

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Neural Regeneration Research 2017年第8期12卷 1278-1286页

作者： Ryo Tamaki Samuel Ige Orie Beat Alessandri Oliver Kempski Axel Heimann Department of Neurosurgery Nara Medical University university Medical Center of the johannes gutenberg-university of mainz Institute for Neurosurgical Pathophysiology

Endogenous neurogenesis can arise from a variety of physiological stimuli including exercise, learning, or ＂enriched environment＂ as well as pathological conditions such as ischemia, epilepsy or cortical spreading depression. Whether all these conditions use a common trigger to set off endogenous neurogenesis is yet unclear. We hypothesized that cortical spreading depression（CSD） induces neurogenesis in the cerebral cortex and dentate gyrus after cerebral venous ischemia. Forty-two Wistar rats alternatively underwent sham operation（Sham）, induction of ten CSDs or venous ischemia provoked via occlusion of two adjacent superficial cortical vein followed by ten induced CSDs（CSD ＋ 2-VO）. As an additional control, 15 na？ve rats received no intervention except 5-bromo-2′-deoxyuridine（Brd U） treatment for 7 days. Sagittal brain slices（40 μm thick） were co-stained for Brd U and doublecortin（DCX; new immature neuronal cells） on day 9 or Neu N（new mature neuronal cells） on day 28. On day 9 after sham operation, cell proliferation and neurogenesis occurred in the cortex in rats. The sole induction of CSD had no effect. But on days 9 and 28, more proliferating cells and newly formed neurons in the ipsilateral cortex were observed in rats subjected to CSD ＋ 2VO than in rats subjected to sham operation. On days 9 and 28, cell proliferation and neurogenesis in the ipsilateral dentate gyrus was increased in sham-operated rats than in na？ve rats. Our data supports the hypothesis that induced cortical neurogenesis after CSD ＋ 2-VO is a direct effect of ischemia, rather than of CSD alone.

关键词： nerve regeneration cortical spreading depression two-vein occlusion adult neurogenesis stem cells cerebral cortex neural precursor cells neuron penumbra neural regeneration

Hepatocellular carcinoma:one world,one cancer—different guidelines?

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Hepatobiliary Surgery and Nutrition 2018年第1期7卷 41-43页

作者： Friedrich Foerster Peter Robert Galle Department of Internal Medicine I University Medical Center of the Johannes Gutenberg-University MainzMainzGermany

Hepatocellular carcinoma(HCC)is a healthcare challenge in virtually all parts of the ***,it is no wonder that different guidelines have been developed reflecting expert consensus on the management of HCC in different regions,namely guidelines from the American Association for the Study of Liver Diseases(AASLD)for the USA(1,2),the Asian Pacific Association for the Study of the Liver(APASL)for the Asia-Pacific region(3),the European Association for the Study of the Liver(EASL)for Europe(4),and the Japan Society of Hepatology(JSH)for Japan(5,6).

关键词： Liver carcinoma cancer

Choroid plexus imaging to track neuroinflammation—a translational model for mouse and human studies

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Neural Regeneration Research 2023年第3期18卷 521-522页

作者： Muthuraman Muthuraman Mohammadsaleh Oshaghi Vinzenz Fleischer Dumitru Ciolac Ahmed Othman Sven G.Meuth Gabriel Gonzalez-Escamilla Sergiu Groppa Department of Neurology Focus Program Translational NeuroscienceRhine Main Neuroscience Network(rmn2)University Medical Center of the Johannes Gutenberg University MainzMainzGermany Department of Neuroradiology University Medical Center of the Johannes Gutenberg University MainzMainzGermany Department of Neurology Medical FacultyHeinrich-Heine-UniversityDüsseldorfGermany

The choroid plexus(ChP)is a highly vascularized and secretory tissue in each of the brain ventricles that represents the key structure between the blood and the cerebrospinal fluid(CSF).Besides its essential role in CSF production and brain waste clearance pathways,the ChP also contributes to the regulation of central nervous system(CNS)immunosurveillance(Ghersi-Egea et al.,2018).Indeed,the ChP forming the blood-CSF barrier(BCSFB)regulates the entry of immune cells and solute molecules into the brain and vice *** antigen-specific,autoreactive immune activation occurs in the periphery,inflammatory cells migrate through the brain barriers towards the CNS(Strominger et al.,2018),initiating neuroinflammatory diseases such as multiple sclerosis(MS).

关键词： al. plexus inflammation