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Showing 1-28 of 28 results for "HPA001401" within Papers
Martin Madill et al.
Molecular brain, 10(1), 22-22 (2017-06-15)
Amyotrophic lateral sclerosis, a devastating neurodegenerative disease, is characterized by the progressive loss of motor neurons and the accumulation of misfolded protein aggregates. The latter suggests impaired proteostasis may be a key factor in disease pathogenesis, though the underlying mechanisms
Mehdi Touat et al.
The Journal of clinical investigation, 128(4), 1671-1687 (2018-02-16)
Synthetic lethality is an efficient mechanism-based approach to selectively target DNA repair defects. Excision repair cross-complementation group 1 (ERCC1) deficiency is frequently found in non-small-cell lung cancer (NSCLC), making this DNA repair protein an attractive target for exploiting synthetic lethal
Tomohiro Maruyama et al.
Cell death & disease, 9(8), 797-797 (2018-07-25)
DDHD2/KIAA0725p is a mammalian intracellular phospholipase A1 that exhibits phospholipase and lipase activities. Mutation of the DDHD2 gene causes hereditary spastic paraplegia (SPG54), an inherited neurological disorder characterized by lower limb spasticity and weakness. Although previous studies demonstrated lipid droplet
Pei-San Huang et al.
Stem cell research, 54, 102415-102415 (2021-06-13)
Superoxide Dismutase 1 (SOD1) is an antioxidant enzyme that protects the cells from radical oxygen species. To study the behavior of endogenous SOD1 under a microscope, we genetically modified H1 human embryonic stem cells (hESCs) to express SOD1 fused with
David Bayer et al.
Translational neurodegeneration, 10(1), 17-17 (2021-06-02)
Increased catabolism has recently been recognized as a clinical manifestation of amyotrophic lateral sclerosis (ALS). The hypothalamic systems have been shown to be involved in the metabolic dysfunction in ALS, but the exact extent of hypothalamic circuit alterations in ALS
Anette Langebäck et al.
Scientific reports, 9(1), 19384-19384 (2019-12-20)
The use of taxanes has for decades been crucial for treatment of several cancers. A major limitation of these therapies is inherent or acquired drug resistance. A key to improved outcome of taxane-based therapies is to develop tools to predict
Yi Hui Yee et al.
Redox biology, 41, 101834-101834 (2021-04-11)
Apart from its physiological role in inflammation and immunity, the nuclear factor-kappa B (NF-κB) protein complex has been implicated in tumorigenesis and its progression. Here, we provide evidence that a pro-oxidant milieu is an upstream effector of oncogenic NF-κB signaling.
Shyh-Ming Yang et al.
Journal of medicinal chemistry, 61(11), 4883-4903 (2018-05-17)
Aldehyde dehydrogenases (ALDHs) are responsible for the metabolism of aldehydes (exogenous and endogenous) and possess vital physiological and toxicological functions in areas such as CNS, inflammation, metabolic disorders, and cancers. Overexpression of certain ALDHs (e.g., ALDH1A1) is an important biomarker
Reactive Astrocytes Promote ALS-like Degeneration and Intracellular Protein Aggregation in Human Motor Neurons by Disrupting Autophagy through TGF-β1.
Tripathi P, et al.
Stem Cell Reports, 9(2), 667-680 (2017)
Two novel mutations in the gene for copper zinc superoxide dismutase in UK families with amyotrophic lateral sclerosis.
Z E Enayat et al.
Human molecular genetics, 4(7), 1239-1240 (1995-07-01)
Teemu P Miettinen et al.
Molecular pharmaceutics, 11(12), 4395-4404 (2014-10-15)
The analgesic and antipyretic compound acetaminophen (paracetamol) is one of the most used drugs worldwide. Acetaminophen overdose is also the most common cause for acute liver toxicity. Here we show that acetaminophen and many structurally related compounds bind quinone reductase
Aberrant association of misfolded SOD1 with Na+/K+ ATPase-alpha3 impairs its activity and contributes to motor neuron vulnerability in ALS
Ruegsegger C, et al.
Acta Neuropathologica, 131(3), 427-451 (2016)
D R Rosen et al.
Nature, 362(6415), 59-62 (1993-03-04)
Amyotrophic lateral sclerosis (ALS) is a degenerative disorder of motor neurons in the cortex, brainstem and spinal cord. Its cause is unknown and it is uniformly fatal, typically within five years. About 10% of cases are inherited as an autosomal
M Kostrzewa et al.
Human genetics, 98(1), 48-50 (1996-07-01)
Mutation analysis of the superoxide dismutase gene SOD1 in a familial case of amyotrophic lateral sclerosis revealed a T --> C transition at codon 151 of exon 5. This mutation results in the substitution of an isoleucine for a threonine.
Lin Sun et al.
The Journal of biological chemistry, 291(11), 5688-5707 (2016-01-23)
Diabetic nephropathy (DN) is characterized by perturbations in metabolic/cellular signaling pathways with generation of reactive oxygen species (ROS). The ROS are regarded as a common denominator of various pathways, and they inflict injury on renal glomerular cells. Recent studies indicate
Caffeine intake decreases oxidative stress and inflammatory biomarkers in experimental liver diseases induced by thioacetamide: Biochemical and histological study
Amer MG, et al.
International Journal of Immunopathology and Pharmacology, 30(1), 13-24 (2017)
SOD1 transcriptional and posttranscriptional regulation and its potential implications in ALS
Milani P, et al.
Neurology research international, 2011`, 52-56 (2011)
Archana Yadav et al.
Neuron, 111(3), 328-344 (2023-02-03)
The mammalian spinal cord functions as a community of cell types for sensory processing, autonomic control, and movement. While animal models have advanced our understanding of spinal cellular diversity, characterizing human biology directly is important to uncover specialized features of
Jakub Treml et al.
Biomolecules, 9(9) (2019-09-12)
The stilbenoids, a group of naturally occurring phenolic compounds, are found in a variety of plants, including some berries that are used as food or for medicinal purposes. They are known to be beneficial for human health as anti-inflammatory, chemopreventive
Kevin Sea et al.
The Journal of biological chemistry, 290(4), 2405-2418 (2014-11-30)
The functional and structural significance of the intrasubunit disulfide bond in copper-zinc superoxide dismutase (SOD1) was studied by characterizing mutant forms of human SOD1 (hSOD) and yeast SOD1 lacking the disulfide bond. We determined x-ray crystal structures of metal-bound and
Sophia Ceder et al.
Cell death & disease, 12(7), 709-709 (2021-07-17)
Asparaginase depletes extracellular asparagine in the blood and is an important treatment for acute lymphoblastic leukemia (ALL) due to asparagine auxotrophy of ALL blasts. Unfortunately, resistance occurs and has been linked to expression of the enzyme asparagine synthetase (ASNS), which
Yasuo Miki et al.
Neuropathology : official journal of the Japanese Society of Neuropathology, 42(3), 204-211 (2022-03-12)
In typical adult neuronal intranuclear inclusion disease (NIID) with predilection for the basal ganglia or cerebral cortex, not only neurons but also glial cells harbor intranuclear inclusions. In addition, these inclusions are present in the peripheral autonomic nervous system, visceral
Pratibha Tripathi et al.
Stem cell reports, 9(2), 667-680 (2017-07-18)
Amyotrophic lateral sclerosis (ALS) is a fatal and rapidly progressing motor neuron disease. Astrocytic factors are known to contribute to motor neuron degeneration and death in ALS. However, the role of astrocyte in promoting motor neuron protein aggregation, a disease
Multiple transcripts of the human Cu, Zn superoxide dismutase gene
Hirano M, et al.
Biochemical and Biophysical Research Communications, 276(1), 52-56 (2000)
Jakub Treml et al.
Molecules (Basel, Switzerland), 26(9) (2021-05-01)
Background: Oxidative stress is a key factor in the pathophysiology of many diseases. This study aimed to verify the antioxidant activity of selected plant phenolics in cell-based assays and determine their direct or indirect effects. Methods: The cellular antioxidant assay
Laura Pintado-Berninches et al.
Cell death and differentiation, 26(10), 1998-2014 (2019-01-24)
Ataxia telangiectasia (AT) is a genetic disease caused by mutations in the ATM gene but the mechanisms underlying AT are not completely understood. Key functions of the ATM protein are to sense and regulate cellular redox status and to transduce
Amaya Rando et al.
PloS one, 14(1), e0210752-e0210752 (2019-01-15)
Amyotrophic lateral sclerosis (ALS) is a lethal motor neuron disease with no cure. Currently there are only two ALS drugs approved by the FDA, both with a limited therapeutic effect. In the search for drug candidates for ALS, we studied
Teemu P Miettinen et al.
The EMBO journal, 37(10) (2018-04-20)
Palbociclib is a CDK4/6 inhibitor approved for metastatic estrogen receptor-positive breast cancer. In addition to G1 cell cycle arrest, palbociclib treatment results in cell senescence, a phenotype that is not readily explained by CDK4/6 inhibition. In order to identify a
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