Results 41 to 50 of about 88,611 (239)

Accumulation of alkyl-lysophosphatidylcholines in Niemann-Pick disease type C1

Journal of Lipid Research
Lysosomal function is impaired in Niemann-Pick disease type C1 (NPC1), a rare and inherited neurodegenerative disorder, resulting in late endosomal/lysosomal accumulation of unesterified cholesterol.
Sonali Mishra, Pamela Kell, David Scherrer, Dennis J. Dietzen, Charles H. Vite, Elizabeth Berry-Kravis, Cristin Davidson, Stephanie M. Cologna, Forbes D. Porter, Daniel S. Ory, Xuntian Jiang   +10 more
doaj   +1 more source

The Grey Zone Project: Risk-Based Classification of ABCD1 Variants in X-Linked Adrenoleukodystrophy. [PDF]

J Inherit Metab Dis
ABSTRACT Newborn screening (NBS) for X‐linked adrenoleukodystrophy (ALD) enables early identification of boys at risk for adrenal insufficiency (AI) and cerebral ALD (CALD). However, NBS frequently identifies ABCD1 variants of uncertain significance (VUS), which are associated with only borderline‐elevated C26:0‐lysophosphatidylcholine (LPC(26:0 ...
Lund TC, Miettunen K, Jaspers YRJ, Bergner C, Bonkowsky JL, Bruschi F, Cohen JS, Dijkstra IME, Eichler FS, Mallack EJ, Salomons GS, Thompson R, Tonduti D, van Haren KP, Wamelink MMC, Zerem A, Engelen M, Kemp S.   +17 more
europepmc   +2 more sources

Automatic Speech Recognition for Indonesian using Linear Predictive Coding (LPC) and Hidden Markov Model (HMM) [PDF]

, 2015
Speech recognition is influential signal processing in communication technology. Speech recognition has allowed software to recognize the spoken word. Automatic speech recognition could be a solution to recognize the spoken word.
Adhy, Satriyo, Akbar, Rizky, Endah, Sukmawati Nur, Sutikno, S.   +3 more
core  

Recursive least squares for online dynamic identification on gas turbine engines [PDF]

, 2016
Online identification for a gas turbine engine is vital for health monitoring and control decisions because the engine electronic control system uses the identified model to analyze the performance for optimization of fuel consumption, a response to ...
Nalianda, Devaiah, Nikolaidis, Theoklis, Zhou, Li   +2 more
core   +1 more source

Targeting the PGRN‐BMP Lysosomal Axis With NPs@PGRN Reverses Immunometabolic Dysfunction in Chronic Septic Arthritis

Advanced Science, EarlyView.
Chronic septic arthritis involves intracellular bacterial persistence and lipid‐immune crosstalk via the PGRN‐BMP lysosomal axis. A dual‐targeting nanoparticle system (NPs@PGRN) restores lysosomal bactericidal function, reduces bacterial burden, and reprograms macrophage immunity, offering a novel therapeutic strategy. ABSTRACT Chronic septic arthritis,
Congsun Li, Jiaqi Fan, Tao Sun, Wushi Cui, Jiaxuan Zou, Weinan Yang, Liang Chen, Jian Xiao, Shicheng Wang, Tao Zhang, Jianqiao Hong, An Liu, Haobo Wu   +12 more
wiley   +1 more source

Lysophosphatidylcholine Activates Extracellular-Signal-Regulated Protein Kinase and Potentiates Vascular Contractile Responses in Rat Aorta

Journal of Pharmacological Sciences, 2003
We previously reported that in the endothelium-denuded rat aorta, lysophosphatidylcholine (LPC) potentiates the contractile responses induced by high-K+, UK14,304 (a selective a2-adrenoceptor agonist), and phorbol ester with an associated tyrosine ...
Hiroshi Suenaga, Katsuo Kamata
doaj   +1 more source

Pulse Shape Analysis and Identification of Multipoint Events in a Large-Volume Proportional Counter in an Experimental Search for 2K Capture Kr-78

, 2010
A pulse shape analysis algorithm and a method for suppressing the noise component of signals from a large copper proportional counter in the experiment aimed at searching for 2K capture of Kr-78 are described. These signals correspond to a compound event
A. M. Gangapshev, A. Savitzky, A.D. Hillery, A.I. Abramov, A.S. Barabash, A.S. Barabash, D. Donoho, D.L. Donoho, E. Storm, F. Abramovich, F.A. Danevich, H.H. Loosli, Ju.M. Gavriljuk, M. Aunola, M. Doi, M. Hirsch, M. Neumann, M.A. Blokhin, M.A. Blokhin, N.I. Rukhadze, O.A. Rumyantsev, R.W. Hamming, S. I. Panasenko, S. Mallat, S. P. Yakimenko, S. S. Ratkevich, S.S. Ratkevich, V. V. Kazalov, V. V. Kuz’minov, V.I. Tretyak, V.V. Kuzminov, W. Sweldens, W.M. Chew, Yu. M. Gavrilyuk   +33 more
core   +1 more source

Endogenous Ceramide 24:1 Constrains Th17‐Driven Neutrophilic Inflammation by Antagonizing EP2 Signaling

Advanced Science, EarlyView.
Cer24:1 levels are reduced in neutrophilic asthma and inversely correlate with disease severity and airway neutrophilia. Restoring Cer24:1 suppresses pathogenic Th17 differentiation by engaging EP2 on CD4+ T cells, thereby dampening the JAK2–STAT3–RORγt axis and reducing IL‐17 production.
Huan Liu, Abudureyimujiang Aili, Zheng Kuang, Liting Cao, Zemin Li, Ying Shang, Yingying Ge, Tingting Hu, Yongchang Sun, Wuli Zhao, Rong Jin, Chun Chang   +11 more
wiley   +1 more source

Phosphatidic Acid‐TRIM59‐Olig2 Signaling Couples Metabolic Dysfunction to Myelination Failure in PWMI

Advanced Science, EarlyView.
PA accumulates after hypoxic‐ischemic injury and stabilizes the E3 ligase TRIM59 in OPCs. Stabilized TRIM59 enhances ubiquitination and degradation of Olig2, blocking differentiation and causing hypomyelination in PWMI. Modulating PA synthesis restores Olig2 levels, improves myelination, and ameliorates behavioral deficits, defining a metabolically ...
Xinyu Li, Yanan Liu, Meng Zhang, Yiwei Liu, Meiting Liu, Kaishen Zhu, Zhian Liu, Chao Ren, Ruiqin Yao   +8 more
wiley   +1 more source

Analysis of a Modern Voice Morphing Approach using Gaussian Mixture Models for Laryngectomees

, 2012
This paper proposes a voice morphing system for people suffering from Laryngectomy, which is the surgical removal of all or part of the larynx or the voice box, particularly performed in cases of laryngeal cancer.
Chadha, Aman, Padhya, Jay, Savardekar, Bharatraaj   +2 more
core   +2 more sources