当前位置： SCI文献检索 > NEUROBIOLOGY OF DISEASE期刊下所有文献 > Huntington's disease of the endocrine pancreas: insulin deficiency and diabetes mellitus due to impaired insulin gene expression.

Huntington's disease of the endocrine pancreas: insulin deficiency and diabetes mellitus due to impaired insulin gene expression.

Abstract：

:In a transgenic mouse model of the neurodegenerative disorder Huntington's disease (HD), age-dependent neurologic defects are accompanied by progressive alterations in glucose tolerance that culminate in the development of diabetes mellitus and insulin deficiency. Pancreatic islets from HD transgenic mice express reduced levels of the pancreatic islet hormones insulin, somatostatin, and glucagon and exhibit intrinsic defects in insulin production. Intranuclear inclusions accumulate with aging in transgenic pancreatic islets, concomitant with the decline in glucose tolerance. HD transgenic mice develop an age-dependent reduction of insulin mRNA expression and diminished expression of key regulators of insulin gene transcription, including the pancreatic homeoprotein PDX-1, E2A proteins, and the coactivators CBP and p300. Disrupted expression of a subset of transcription factors in pancreatic beta cells by a polyglutamine expansion tract in the huntingtin protein selectively impairs insulin gene expression to result in insulin deficiency and diabetes. Selective dysregulation of gene expression in triplet repeat disorders provides a mechanism for pleiotropic cellular dysfunction that restricts the toxicity of ubiquitously expressed proteins to highly specialized subpopulations of cells.

journal_name

Neurobiol Dis

journal_title

Neurobiology of disease

authors

Andreassen OA,Dedeoglu A,Stanojevic V,Hughes DB,Browne SE,Leech CA,Ferrante RJ,Habener JF,Beal MF,Thomas MK

doi

10.1006/nbdi.2002.0562

keywords:

subject

Has Abstract

pub_date

2002-12-01 00:00:00

pages

410-24

issue

3

eissn

0969-9961

issn

1095-953X

pii

S0969996102905620

journal_volume

11

pub_type

杂志文章

相关文献

NEUROBIOLOGY OF DISEASE文献大全
  • Vascular tight junction disruption and angiogenesis in spontaneously hypertensive rat with neuroinflammatory white matter injury.

    abstract：:Vascular cognitive impairment is a major cause of dementia caused by chronic hypoxia, producing progressive damage to white matter (WM) secondary to blood-brain barrier (BBB) opening and vascular dysfunction. Tight junction proteins (TJPs), which maintain BBB integrity, are lost in acute ischemia. Although angiogenesi...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2018.02.012

    authors： Yang Y,Kimura-Ohba S,Thompson JF,Salayandia VM,Cossé M,Raz L,Jalal FY,Rosenberg GA

    更新日期：2018-06-01 00:00:00

  • Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models.

    abstract：:Huntington's disease (HD) is one of the few neurodegenerative diseases with a known genetic cause, knowledge that has enabled the creation of animal models using genetic manipulations that aim to recapitulate HD pathology. The study of behavioral and neuropathological phenotypes of these HD models, however, has been p...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2009.05.007

    authors： Menalled L,El-Khodor BF,Patry M,Suárez-Fariñas M,Orenstein SJ,Zahasky B,Leahy C,Wheeler V,Yang XW,MacDonald M,Morton AJ,Bates G,Leeds J,Park L,Howland D,Signer E,Tobin A,Brunner D

    更新日期：2009-09-01 00:00:00

  • Death of cortical and striatal neurons induced by mitochondrial defect involves differential molecular mechanisms.

    abstract：:An important aspect of Huntington's disease (HD) pathogenesis which may have important therapeutic implications is that the cellular events leading to cell death may be different in cortical and striatal neurons. In the present study, we characterized cellular changes in cortical and striatal neurons treated with the ...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2003.09.013

    authors： Galas MC,Bizat N,Cuvelier L,Bantubungi K,Brouillet E,Schiffmann SN,Blum D

    更新日期：2004-02-01 00:00:00

  • Local insulin and the rapid regrowth of diabetic epidermal axons.

    abstract：:Insulin deficiency may contribute toward the neurological deficits of diabetic polyneuropathy (DPN). In particular, the unique trophic properties of insulin, acting on sensory neuron and axon receptors offer an approach toward reversing loss of skin axons that develops during diabetes. Here we examined how local cutan...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2011.04.012

    authors： Guo G,Kan M,Martinez JA,Zochodne DW

    更新日期：2011-08-01 00:00:00

  • Early and brain region-specific decrease of de novo cholesterol biosynthesis in Huntington's disease: A cross-validation study in Q175 knock-in mice.

    abstract：:Cholesterol precursors and cholesterol levels are reduced in brain regions of Huntington's disease (HD) mice. Here we quantified the rate of in vivo de novo cholesterol biosynthesis in the HD brain. Samples from different brain regions and blood of the heterozygous knock-in mouse model carrying 175 CAG repeats (Q175) ...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2016.11.013

    authors： Shankaran M,Di Paolo E,Leoni V,Caccia C,Ferrari Bardile C,Mohammed H,Di Donato S,Kwak S,Marchionini D,Turner S,Cattaneo E,Valenza M

    更新日期：2017-02-01 00:00:00

  • Identification of an acute functional cross-talk between amyloid-β and glucocorticoid receptors at hippocampal excitatory synapses.

    abstract：:Amyloid-β is a peptide released by synapses in physiological conditions and its pathological accumulation in brain structures necessary for memory processing represents a key toxic hallmark underlying Alzheimer's disease. The oligomeric form of Amyloid-β (Aβο) is now believed to represent the main Amyloid-β species af...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2018.07.001

    authors： Kootar S,Frandemiche ML,Dhib G,Mouska X,Lorivel T,Poupon-Silvestre G,Hunt H,Tronche F,Bethus I,Barik J,Marie H

    更新日期：2018-10-01 00:00:00

  • Genetic interaction between expanded murine Hdh alleles and p53 reveal deleterious effects of p53 on Huntington's disease pathogenesis.

    abstract：:Huntingtin, the protein product of the Huntington's disease (HD) gene, is known to interact with the tumor suppressor p53. It has recently been shown that activation of p53 upregulates the level of huntingtin, both in vitro and in vivo, whereas p53 deficiency in HD-transgenic flies and mice has been found to be benefi...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2006.08.002

    authors： Ryan AB,Zeitlin SO,Scrable H

    更新日期：2006-11-01 00:00:00

  • Oxidative stress increases expression and activity of BACE in NT2 neurons.

    abstract：:Recently an aspartyl protease with beta-secretase activity called BACE was identified. In the present paper we showed that BACE is modulated by the oxidative stress product 4-hydroxynonenal (HNE). Exposure of NT(2) neurons to the two classical pro-oxidant stimuli ascorbate/FeSO(4) and H(2)O(2)/FeSO(4) resulted in a si...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1006/nbdi.2002.0515

    authors： Tamagno E,Bardini P,Obbili A,Vitali A,Borghi R,Zaccheo D,Pronzato MA,Danni O,Smith MA,Perry G,Tabaton M

    更新日期：2002-08-01 00:00:00

  • Pathological remodelling of colonic wall following dopaminergic nigrostriatal neurodegeneration.

    abstract：BACKGROUND AND AIM:Patients with Parkinson's disease (PD) are often characterized by functional gastrointestinal disorders. Such disturbances can occur at all stages of PD and precede the typical motor symptoms of the disease by many years. However, the morphological alterations associated with intestinal disturbances ...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2020.104821

    authors： Pellegrini C,Ippolito C,Segnani C,Dolfi A,Errede M,Virgintino D,Fornai M,Antonioli L,Garelli F,Nericcio A,Colucci R,Cerri S,Blandini F,Blandizzi C,Bernardini N

    更新日期：2020-06-01 00:00:00

  • Regulatory T cells promote remyelination in the murine experimental autoimmune encephalomyelitis model of multiple sclerosis following human neural stem cell transplant.

    abstract：:Multiple sclerosis (MS) is a chronic, inflammatory autoimmune disease that affects the central nervous system (CNS) for which there is no cure. In MS, encephalitogenic T cells infiltrate the CNS causing demyelination and neuroinflammation; however, little is known about the role of regulatory T cells (Tregs) in CNS ti...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2020.104868

    authors： McIntyre LL,Greilach SA,Othy S,Sears-Kraxberger I,Wi B,Ayala-Angulo J,Vu E,Pham Q,Silva J,Dang K,Rezk F,Steward O,Cahalan MD,Lane TE,Walsh CM

    更新日期：2020-07-01 00:00:00

  • Functional genomics, genetic risk profiling and cell phenotypes in neurodegenerative disease.

    abstract：:Human genetics provides unbiased insights into the causes of human disease, which can be used to create a foundation for effective ways to more accurately diagnose patients, stratify patients for more successful clinical trials, discover and develop new therapies, and ultimately help patients choose the safest and mos...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章,评审

    doi：10.1016/j.nbd.2020.105088

    authors： Finkbeiner S

    更新日期：2020-12-01 00:00:00

  • Adenovirus-mediated GDNF and CNTF pretreatment protects against striatal injury following transient middle cerebral artery occlusion in mice.

    abstract：:During the last few years, adenoviral gene transfer techniques have achieved increasing interest in the treatment of neurodegenerative diseases. However, gene therapy requires that delivered genes are translated into proteins. This may pose a problem in focal ischemia where protein synthesis is compromized. The presen...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1006/nbdi.2001.0399

    authors： Hermann DM,Kilic E,Kügler S,Isenmann S,Bähr M

    更新日期：2001-08-01 00:00:00

  • Subtle microstructural changes of the striatum in a DYT1 knock-in mouse model of dystonia.

    abstract：:The dystonias are comprised of a group of disorders that share common neurological abnormalities of involuntary twisting or repetitive movements and postures. The most common inherited primary dystonia is DYT1 dystonia, which is due to loss of a GAG codon in the TOR1A gene that encodes torsinA. Autopsy studies of brai...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2013.01.008

    authors： Song CH,Bernhard D,Bolarinwa C,Hess EJ,Smith Y,Jinnah HA

    更新日期：2013-06-01 00:00:00

  • Phenothiazines interfere with dopaminergic neurodegeneration in Caenorhabditis elegans models of Parkinson's disease.

    abstract：:Oxidative stress is involved in the pathogenesis of various neurodegenerative disorders, conventional antioxidant strategies have yet been of limited success. We have employed transgenic Caenorhabditis elegans expressing DsRed2 in dopaminergic neurons and CFP pan-neuronally, to characterize in larval and adult animals...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2010.03.019

    authors： Mocko JB,Kern A,Moosmann B,Behl C,Hajieva P

    更新日期：2010-10-01 00:00:00

  • The dynamics of cortico-thalamo-cortical interactions at the transition from pre-ictal to ictal LFPs in absence epilepsy.

    abstract：PURPOSE:Generalized spike and wave discharges (SWD) are generated within the cortico-thalamo-cortical system. However the exact interactions between cortex and different thalamic nuclei needed for the generation and maintenance of SWD are still to be elucidated. This study aims to shed more light on these interactions ...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2012.03.023

    authors： Lüttjohann A,van Luijtelaar G

    更新日期：2012-07-01 00:00:00

  • Interneuronal calcium channel abnormalities in posttraumatic epileptogenic neocortex.

    abstract：:Decreased release probability (Pr) and increased failure rate for monosynaptic inhibitory postsynaptic currents (IPSCs) indicate abnormalities in presynaptic inhibitory terminals on pyramidal (Pyr) neurons of the undercut (UC) model of posttraumatic epileptogenesis. These indices of inhibition are normalized in high [...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2011.11.006

    authors： Faria LC,Parada I,Prince DA

    更新日期：2012-02-01 00:00:00

  • Over-expression of tau results in defective synaptic transmission in Drosophila neuromuscular junctions.

    abstract：:We have shown that over-expression of human tau (0N3R) in Drosophila larval motor neurons causes significant morphological and functional disruption to the neuromuscular junctions (NMJs). Tau-expressing NMJs are reduced in size with irregular and abnormal bouton structure. Immunocytochemical analysis shows that the ab...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2005.05.029

    authors： Chee FC,Mudher A,Cuttle MF,Newman TA,MacKay D,Lovestone S,Shepherd D

    更新日期：2005-12-01 00:00:00

  • A double point mutation in the DNA-binding region of Egr2 switches its function from inhibition to induction of proliferation: A potential contribution to the development of congenital hypomyelinating neuropathy.

    abstract：:Mutations in the DNA-binding domain of EGR2 are associated with severe autosomal dominant forms of peripheral neuropathy. In this study, we show that one such Egr2 mutant (S382R, D383Y), when expressed in Schwann cells in vitro, is not transcriptionally inactive but retains residual wild-type Egr2 functions, including...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2006.06.006

    authors： Arthur-Farraj P,Mirsky R,Parkinson DB,Jessen KR

    更新日期：2006-10-01 00:00:00

  • Complement-receptor-3 and scavenger-receptor-AI/II mediated myelin phagocytosis in microglia and macrophages.

    abstract：:Microglia and macrophages express the alpha(M)/beta(2) integrin complement-receptor-3 (CR3/MAC-1; CD11b/CD18) and scavenger-receptor-AI/II (SRAI/II). Both can mediate myelin phagocytosis. We document that CR3/MAC-1 mediated myelin phagocytosis in microglia is modulated by complement and anti-CR3/MAC-1 mAbs. Complement...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/s0969-9961(02)00008-6

    authors： Reichert F,Rotshenker S

    更新日期：2003-02-01 00:00:00

  • RIP3 participates in early brain injury after experimental subarachnoid hemorrhage in rats by inducing necroptosis.

    abstract：:Necroptosis is a regulated form of necrosis that is mediated by a variety of proteins including tumor necrosis factor-α (TNF-α) and receptor-interacting proteins (RIPs). TNF-α, a critical inflammatory molecule, is one of the initiating signals in the necroptosis pathway, and RIP3 acts as a switch that commits the cell...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2019.05.004

    authors： Yuan S,Yu Z,Zhang Z,Zhang J,Zhang P,Li X,Li H,Shen H,Chen G

    更新日期：2019-09-01 00:00:00

  • AGS-induced expression of Narp is concomitant with expression of AMPA receptor subunits GluR1 and GluR2 in hippocampus but not inferior colliculus of P77PMC rats.

    abstract：:To explore mechanisms of epileptogenesis in audiogenic seizures (AGS), we examined the expression of alpha-amino-3-hydroxy-5-methyl-4-isoxazoleopropionic acid (AMPA) receptor subunits GluR1 and GluR2 and of the GluR-associated protein Narp in the hippocampus and the inferior colliculus (IC) from AGS-susceptible P77PMC...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2003.08.010

    authors： Li SY,Xu DS,Jia HT

    更新日期：2003-12-01 00:00:00

  • Autism with seizures and intellectual disability: possible causative role of gain-of-function of the inwardly-rectifying K+ channel Kir4.1.

    abstract：:The inwardly-rectifying potassium channel Kir4.1 is a major player in the astrocyte-mediated regulation of [K(+)](o) in the brain, which is essential for normal neuronal activity and synaptic functioning. KCNJ10, encoding Kir4.1, has been recently linked to seizure susceptibility in humans and mice, and is a possible ...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2011.03.016

    authors： Sicca F,Imbrici P,D'Adamo MC,Moro F,Bonatti F,Brovedani P,Grottesi A,Guerrini R,Masi G,Santorelli FM,Pessia M

    更新日期：2011-07-01 00:00:00

  • Identification of a unique apolipoprotein E allele in Microcebus murinus; ApoE brain distribution and co-localization with beta-amyloid and tau proteins.

    abstract：:This report is devoted to the characterization of the apolipoprotein E (ApoE) in Microcebus murinus. Only one allele homologous to the human ApoE4 allele was evidenced. The distribution of the corresponding ApoE protein in the brain was found in association with the pathological proteins characteristic of Alzheimer's ...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1006/nbdi.1995.0018

    authors： Calenda A,Jallageas V,Silhol S,Bellis M,Bons N

    更新日期：1995-06-01 00:00:00

  • N-Palmitoylethanolamine-oxazoline (PEA-OXA): A new therapeutic strategy to reduce neuroinflammation, oxidative stress associated to vascular dementia in an experimental model of repeated bilateral common carotid arteries occlusion.

    abstract：AIM:Recent studies revealed that pharmacological modulation of NAE-hydrolyzing acid amidase (NAAA) can be achieved with PEA oxazoline (PEA-OXA). Hence, the aim of the present work was to thoroughly evaluate the anti-inflammatory and neuroprotective effects of PEA-OXA in an experimental model of vascular dementia (VaD) ...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2019.01.007

    authors： Impellizzeri D,Siracusa R,Cordaro M,Crupi R,Peritore AF,Gugliandolo E,D'Amico R,Petrosino S,Evangelista M,Di Paola R,Cuzzocrea S

    更新日期：2019-05-01 00:00:00

  • Chronic minocycline treatment improves hippocampal neuronal structure, NMDA receptor function, and memory processing in Fmr1 knockout mice.

    abstract：:Fragile X Syndrome (FXS) is the most common inherited cause of intellectual disability, and is the leading known single-gene cause of autism spectrum disorder. FXS patients display varied behavioural deficits that include mild to severe cognitive impairments in addition to mood disorders. Currently there is no cure fo...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2018.01.014

    authors： Yau SY,Bettio L,Vetrici M,Truesdell A,Chiu C,Chiu J,Truesdell E,Christie BR

    更新日期：2018-05-01 00:00:00

  • α-Synuclein-mediated inhibition of ATF6 processing into COPII vesicles disrupts UPR signaling in Parkinson's disease.

    abstract：:The unfolded protein response (UPR) monitors the folding environment within the endoplasmic reticulum (ER). Accumulation of misfolded proteins within the ER activates the UPR resulting in the execution of adaptive or non-adaptive signaling pathways. α-Synuclein (α-syn) whose accumulation and aggregation define the pat...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2015.02.005

    authors： Credle JJ,Forcelli PA,Delannoy M,Oaks AW,Permaul E,Berry DL,Duka V,Wills J,Sidhu A

    更新日期：2015-04-01 00:00:00

  • The SARS-CoV-2 spike protein alters barrier function in 2D static and 3D microfluidic in-vitro models of the human blood-brain barrier.

    abstract：:As researchers across the globe have focused their attention on understanding SARS-CoV-2, the picture that is emerging is that of a virus that has serious effects on the vasculature in multiple organ systems including the cerebral vasculature. Observed effects on the central nervous system include neurological symptom...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2020.105131

    authors： Buzhdygan TP,DeOre BJ,Baldwin-Leclair A,Bullock TA,McGary HM,Khan JA,Razmpour R,Hale JF,Galie PA,Potula R,Andrews AM,Ramirez SH

    更新日期：2020-12-01 00:00:00

  • Differential CSF butyrylcholinesterase levels in Alzheimer's disease patients with the ApoE epsilon4 allele, in relation to cognitive function and cerebral glucose metabolism.

    abstract：:Butyrylcholinesterase (BuChE) is increased in the cerebral cortex of Alzheimer's disease (AD) patients, particularly those carrying epsilon4 allele of the apolipoprotein E gene (ApoE) and certain BuChE variants that predict increased AD risk and poor response to anticholinesterase therapy. We measured BuChE activity a...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2006.07.013

    authors： Darreh-Shori T,Brimijoin S,Kadir A,Almkvist O,Nordberg A

    更新日期：2006-11-01 00:00:00

  • PTEN deletion increases hippocampal granule cell excitability in male and female mice.

    abstract：:Deletion of the mTOR pathway inhibitor PTEN from postnatally-generated hippocampal dentate granule cells causes epilepsy. Here, we conducted field potential, whole cell recording and single cell morphology studies to begin to elucidate the mechanisms by which granule cell-specific PTEN-loss produces disease. Cells fro...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2017.08.014

    authors： Santos VR,Pun RYK,Arafa SR,LaSarge CL,Rowley S,Khademi S,Bouley T,Holland KD,Garcia-Cairasco N,Danzer SC

    更新日期：2017-12-01 00:00:00

  • Reducing canonical Wingless/Wnt signaling pathway confers protection against mutant Huntingtin toxicity in Drosophila.

    abstract：:Huntington's disease (HD) is a genetic neurodegenerative disease characterized by movement disorders, cognitive decline and neuropsychiatric symptoms. HD is caused by expanded CAG tract within the coding region of Huntingtin protein. Despite major insights into the molecular mechanisms leading to HD, no effective cure...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2012.04.007

    authors： Dupont P,Besson MT,Devaux J,Liévens JC

    更新日期：2012-08-01 00:00:00