Neurodegeneration: Causes & Reasons

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Possible Causes for neurodegeneration

Vitamin B12 Deficiency

[…] on cognitive function, this study lends support to the notion that high serum folate concentrations in older people with VB12 deficiency may be associated with increased neurodegeneration [ncbi.nlm.nih.gov]

Oxidative stress is implicated in various human diseases and conditions, such as a neurodegeneration, which is the major symptom of vitamin B 12 deficiency, although the underlying [ncbi.nlm.nih.gov]

Neurodegeneration in methylmalonic aciduria involves inhibition of complex II and the tricarboxylic acid cycle, and synergistically acting excitotoxicity. [doi.org]

Alzheimer Disease

Furthermore, in DLB, Aβ 1–42 may promote α-synuclein accumulation and neurodegeneration. [dx.doi.org]

A. (2003) Alpha-synuclein overexpression protects against paraquat-induced neurodegeneration. J. Neurosci. 23, 3095–3099. [dx.doi.org]

Pallidopyramidal Syndrome

AB - Kufor-Rakeb syndrome is an autosomal recessive nigro-striatal-pallidal-pyramidal neurodegeneration. [research.manchester.ac.uk]

Understanding how FBXO7 mutations cause disease will shed further light on the molecular mechanisms of neurodegeneration, with potential implications also for more common [moh-it.pure.elsevier.com]

Conclusions: We conclusively show that recessive FBXO7 mutations cause progressive neurodegeneration with extrapyramidal and pyramidal system involvement, delineating a novel [n.neurology.org]

Parkinson Disease

In this review, we address the current concept of neuroinflammation and its involvement in PD-associated neurodegeneration. [ncbi.nlm.nih.gov]

However, 5-10% of PD cases are inherited as monogenic diseases, which provides a chance to understand the molecular mechanisms underlying neurodegeneration. [ncbi.nlm.nih.gov]

Open Access Review 1 Department of Biological Sciences, The University of Alabama, Tuscaloosa, AL 35487, USA 2 Departments of Neurology and Neurobiology, Center for Neurodegeneration [doi.org]

Lafora Disease

These animals did not show the increase in markers of neurodegeneration, the impairments in electrophysiological properties of hippocampal synapses, nor the susceptibility [ncbi.nlm.nih.gov]

Lafora disease is a metabolic disorder resulting in accumulation of water-insoluble glucan in the cytoplasm, and manifests as a debilitating neurodegeneration that ends with [ncbi.nlm.nih.gov]

Our results emphasize the relevance of the laforin-malin complex in the control of glycogen metabolism and highlight altered glycogen accumulation as a key contributor to neurodegeneration [ncbi.nlm.nih.gov]

X-linked Parkinsonism-Spasticity Syndrome

Test Registry Neurodegeneration With Brain Iron Accumulation 3 Basal Ganglia Disease, Adult-Onset Neuroferritinopathy NBIA3 606159 Genetic Test Registry Neurodegeneration [ukgtn.nhs.uk]

MeSH Descriptor Source: MeSH 2019 deskriptory Main Heading Hallervordenův-Spatzův syndrom English Heading Pantothenate Kinase-Associated Neurodegeneration See (Czech) neurodegenerace [medvik.cz]

Amyotrophic lateral sclerosis, susceptibility to DNAJC6 Parkinson disease 19a, juvenile-onset EIF4G1 Parkinson disease 18 FBXO7 Parkinson disease 15, autosomal recessive FTL Neurodegeneration [asperbio.com]

Wolfram Syndrome

This work is necessary to position the field for future clinical trials to test interventions for WFS neurodegeneration. [clinicaltrials.gov]

However, the mechanisms underlying neurodegeneration caused by WFS1 deficiency remain elusive. [ncbi.nlm.nih.gov]

In patients with Wolfram syndrome, these mechanisms underlie progressive neurodegeneration and endocrine dysfunction. [care.diabetesjournals.org]

Progressive Myoclonic Epilepsy Type 3

Neurodegeneration and regression occur with seizure onset. Other features include mental retardation, dysarthria, truncal ataxia, and loss of fine finger movements. [malacards.org]

Symptoms The list of signs and symptoms mentioned in various sources for Epilepsy, progressive myoclonic 3 includes the 6 symptoms listed below: * Myoclonic epilepsy * Neurodegeneration [checkorphan.org]

[…] myoclonic type 3 The list of signs and symptoms mentioned in various sources for Epilepsy, progressive myoclonic 3 includes the 6 symptoms listed below: * Myoclonic epilepsy * Neurodegeneration [checkorphan.org]

Primary Progressive Multiple Sclerosis

Spinal neurodegeneration is an important determinant of disability progression in patients with primary progressive multiple sclerosis. [ncbi.nlm.nih.gov]

The effect of current immunomodulatory treatments on neurodegeneration is insufficient. [ncbi.nlm.nih.gov]

Studies on the histology and pathogenesis show that neurodegeneration is predominant over inflammation in PPMS, even if cellular and humoral immune-mediated mechanisms are [ncbi.nlm.nih.gov]

Spinal and Bulbar Muscular Atrophy

Pre-clinical studies using animal models show that the pathogenic AR-mediated neurodegeneration is suppressed by androgen inactivation, the efficacy of which has been tested [ncbi.nlm.nih.gov]

Animal studies have revealed that the pathogenesis of SBMA depends on the level of serum testosterone, and that androgen deprivation mitigates neurodegeneration through inhibition [ncbi.nlm.nih.gov]

Abstract Expanded polyglutamine tracts cause neurodegeneration through a toxic gain-of-function mechanism. [ncbi.nlm.nih.gov]

Progressive Myoclonic Epilepsy Type 8

Knockdown of Cers1 in mouse neuroblastoma cells triggered endoplasmic reticulum stress, the unfolded protein response, and apoptosis, suggesting a mechanism for neurodegeneration [onlinelibrary.wiley.com]

[…] levels of ceramides and sphingolipids containing very long‐chain fatty acids have been associated with many neurodegenerative disorders, including cerebellar Purkinje cell neurodegeneration [onlinelibrary.wiley.com]

Hallervorden-Spatz Syndrome

(disorder) Pigmentary pallidal degeneration Neurodegeneration With Brain Iron Accumulation type 1 Pantothenate Kinase-Associated Neurodegeneration NEURODEGENERATION WITH [wikidata.org]

neurodegeneration; PKAN pantothenate kinase-associated neurodegeneration; XL X-linked 1. [ncbi.nlm.nih.gov]

BRAIN IRON ACCUMULATION 1; NBIA1 PKAN Pkan Neuroaxonal Dystrophy, Juvenile-Onset edit English pantothenate kinase-associated neurodegeneration A neurodegeneration with brain [wikidata.org]

Amyotrophic Lateral Sclerosis

Here we review some of the recent progress in promoting therapeutic strategies for neurodegeneration. [doi.org]

This review aims to investigate how oxidative stress can affect other proposed mechanisms of neurodegeneration in ALS. [ncbi.nlm.nih.gov]

Strategies for clinical approach to neurodegeneration in amyotrophic lateral sclerosis Cecilia Carlesi, Livia Pasquali, Selina Piazza, Annalisa Lo Gerfo, Elena Caldarazzo [doi.org]

Neuronal Ceroid Lipofuscinosis

Thus, we sought to determine whether this pathway of apoptosis contributes to rapid neurodegeneration in INCL. [doi.org]

Mutant dnj-14 worms also exhibited age-dependent neurodegeneration of sensory neurons, which was preceded by severe progressive chemosensory defects. [ncbi.nlm.nih.gov]

Autosomal-dominant adult-onset neuronal ceroid lipofuscinosis (ANCL) is characterized by accumulation of autofluorescent storage material in neural tissues and neurodegeneration [ncbi.nlm.nih.gov]

McLeod Neuroacanthocytosis Syndrome

The mechanism by which these mutations cause neurodegeneration is not known. dcterms:identifier kegg:H00832 void:inDataset Bio2RDF identifier H00832 Bio2RDF namespace kegg [bio2rdf.org]

HARP syndrome is allelic with pantothenate kinase-associated neurodegeneration. [ncbi.nlm.nih.gov]

The mechanism by which these mutations cause neurodegeneration is not known. [genome.jp]

REM Sleep Behavior Disorder

So far, the number of studies assessing predictive markers of neurodegeneration in RBD is limited. [n.neurology.org]

[…] treatment consequences of RBD in the context of neurodegeneration. 2018 S. Karger AG, Basel. [ncbi.nlm.nih.gov]

"Rapid-eye movement sleep behavior disorder is not just a precursor but also a critical warning sign of neurodegeneration that can lead to brain disease," John Peever, associate [universityherald.com]

Progressive Supranuclear Palsy

CurePSP CurePSP is the leading source of information and support for patients and their families, other caregivers, researchers, and healthcare professionals on prime of life neurodegeneration [psp.org]

Neurodegeneration: the molecular pathology of dementia and movement disorders. Basel : ISN Neuropath Press ; 2003. p. 103 – 14. 5. [doi.org]

Clinical and neuropathological correlations in atypical neurodegenerations are crucial to describe new entities of overlapping syndromes. [ncbi.nlm.nih.gov]

Huntington Disease

In this review we consider the current status of research in the field and possible mechanisms whereby the HD mutation might result in neurodegeneration. [ncbi.nlm.nih.gov]

Regulation of autophagy by polyphenols: Paving the road for treatment of neurodegeneration. Biotechnology Advances, Vol. 36, Issue. 6, p. 1768. [dx.doi.org]

Neurodegenerative Disorder

In addition, it is the progressive neurodegeneration which determines the amount of accumulating clinical disability. [ncbi.nlm.nih.gov]

[…] and Repair: The interdepartmental program in Cellular Neuroscience, Neurodegeneration and Repair (CNNR) will accelerate the pace of Yale's research on neurodegeneration diseases [medicine.yale.edu]

Neurodegeneration is the overarching term for conditions with progressive loss of function and eventually death of neurons participating in motor, sensory or cognitive functions [ki.se]

Motor Neuron Disease

Neurodegeneration 1995 ; 4 : 209 –16. Spreux-Varoquaux O, Bensimon G, Lacomblez L, et al. [doi.org]

Renaud -- Neurodegeneration with brain iron accumulation, Wilson disease, manganism / Alisdair McNeill. [worldcat.org]

[…] cellular signature of aggregated TDP-43 common to nearly all MND and a large proportion of frontotemporal dementia (FTD), has placed MND alongside more traditional cerebral neurodegeneration [ncbi.nlm.nih.gov]

Classic Progressive Supranuclear Palsy Syndrome

The factors that initiate tau-neurodegeneration are unknown. Diagnostic methods Diagnosis is based on the clinical picture and neuropsychological evaluation. [orpha.net]

Parkinson's Disease Type 3

[…] hearing loss; Alternating hemiplegia of childhood 2 AD 12 ATP6AP2 300556 syndromic mental retardation, Hedera type XLR 2 ATP7B 606882 Wilson disease AR 25 C19orf12 614297 neurodegeneration [centogene.com]

The current medications of the disease cure the symptoms; none of them stops neurodegeneration. [journalmeddbu.com]

On the other hand, pathogenic mutations alone may be sufficient to provoke neurodegeneration. [symptoma.com]

Progressive Myoclonic Epilepsy Type 7

Ataxia-telangiectasia-like disorder Atherosclerosis-deafness-diabetes-epilepsy-nephropathy syndrome Atypical juvenile parkinsonism Atypical pantothenate kinase-associated neurodegeneration [se-atlas.de]

Despite increasing knowledge of the etiology of most progressive myoclonus epilepsy disorders, the pathogenic mechanisms leading to neurodegeneration and epilepsy remain largely [medlink.com]

Neurodegeneration and regression occur with seizure onset. Other features include mental retardation, dysarthria, truncal ataxia, and loss of fine finger movements. [genecards.org]

Machado-Joseph Disease

namely at early stages of another polyglutamine-related disorder such as Huntington's disease, we now tested their ability to control MJD-associated neurodegeneration. [ncbi.nlm.nih.gov]

Selective neurodegeneration in MJD is evident in several subcortical brain regions including the cerebellum. [ncbi.nlm.nih.gov]

Since these findings were detected both by lifetime images and by postmortem examination, MRI intensity changes could track the progression of neurodegeneration. [ncbi.nlm.nih.gov]

Menkes Disease

These diseases are characterized by neurological phenotypes ranging from intellectual disability to neurodegeneration. [ncbi.nlm.nih.gov]

Currently, no therapy effectively arrests the relentless neurodegeneration of Menkes' disease. [ncbi.nlm.nih.gov]

Menkes disease is an X-linked genetic disorder of copper transport that results in death from severe progressive neurodegeneration by the age of 3 years. [ncbi.nlm.nih.gov]

Spinocerebellar Ataxia Type 1

The objective of this study is to evaluate neurochemical changes associated with neurodegeneration in cerebral tissue in SCA1 patients compared to age- and gender-matched [ncbi.nlm.nih.gov]

Keywords Neurodegeneration Polyglutamine Therapeutic approaches References 1. [doi.org]

These data demonstrate that presymptomatic and progressive neurodegeneration in SCA1 can be noninvasively monitored using MRS. [ncbi.nlm.nih.gov]

Gordon-Holmes Syndrome

KEYWORDS: Ataxia; CHIP; Dementia; Early onset ataxia; Early-onset dementia; Gordon Holmes syndrome; Hypogonadism; Magnetic resonance imaging; Neurodegeneration; Neurodegenerative [ncbi.nlm.nih.gov]

STUB1/CHIP mutations cause Gordon Holmes syndrome as part of a widespread multisystemic neurodegeneration: evidence from four novel mutations. [miami.pure.elsevier.com]

STUB1/CHIP mutations cause Gordon Holmes syndrome as part of widespread multisystemic neurodegeneration: Evidence from novel mutations [abstract]. [mdsabstracts.org]

Ataxia Telangiectasia

Our results define a crucial role of the cellular localization of HDAC4 in the events leading to ataxia telangiectasia neurodegeneration. [ncbi.nlm.nih.gov]

The symptoms of ataxia-telangiectasia (A-T) include a progressive neurodegeneration caused by ATM protein deficiency. [ncbi.nlm.nih.gov]

Abstract The symptoms of ataxia-telangiectasia (A-T) include a progressive neurodegeneration caused by ATM protein deficiency. [doi.org]

Neonatal Adrenoleukodystrophy

The childhood form of the disease is quite devastating as the primary damage is of the form of a progressive neurodegeneration with symptoms that expand from mild to severe [ald.org.nz]

This form of ALD usually progresses rapidly, causes neurodegeneration, and results in death in infancy or young childhood. [healthprioritiesinc.com]

Niemann-Pick Disease

Oral administration of SAHA prevents oxidative stress and neurodegeneration, and improves behavioral performance in ASMko mice. [ncbi.nlm.nih.gov]

Hepatic NPC1 re-expression did not ameliorate the onset and progression of neurodegeneration of the NPC1-null animal. [ncbi.nlm.nih.gov]

Based on immunohistochemical evaluation of the olfactory epithelium, we analyzed the impact of neurodegeneration in the olfactory epithelium of NPC1 -/- mice and observed [ncbi.nlm.nih.gov]