Results 101 to 110 of about 6,268,347 (286)

AI‐Assisted Engineering of Glycyrrhizic Acid/Simvastatin Nanocrystals for Multifunctional Treatment of Bacterial Osteomyelitis

Advanced Science, EarlyView.
Through AI‐assisted screening from FDA‐approved API to overcome the limitations of bacterial osteomyelitis treatment, glycyrrhizic acid and simvastatin are identified as a multifunctional combination capable of self‐assembling into mechanism‐targeting nanocrystals that effectively neutralize reactive oxygen species, suppress M1 macrophage polarization,
Yu Han, Yao Zhao, Chengbo Yang, Miao Niu, Jun Dai, Quanxin Ning, Kai Xiao, Jinzheng Liang, Wensheng Zhang, Liang Wang, Dan Shao, Dongsong Li   +11 more
wiley   +1 more source

Cardiac Affection in Lysosomal Storage Disorders: Review Article [PDF]

Zagazig University Medical Journal
A class of illnesses known as lysosomal storage disorders is brought on by deficiencies in membrane transporters, lysosomal enzymes, or other proteins important in lysosomal biology.
Wessam Mokhtar, Nariman Omar, Asmaa Mohammed Niazy, AL Shaymaa Ahmed Ali   +3 more
doaj   +1 more source

Altered cerebellar granule cell differentiation and synapse maturation in a mouse model of a lysosomal lipid storage disease.

, 2020
The rare Niemann-Pick type C1 (NPC1) disease is a lysosomal lipid storage disorder, caused by mutations in the Npc1 gene. Since the encoded protein mediates the outflow of cholesterol from endosomal-lysosomal compartments, these mutations cause ...
Sonia Canterini, Serena Camuso, Micaela Lucarelli, Alessandro Rava, Lucy Babicola, Fiorenza Maria Teresa   +5 more
core  

SPSB1 Promotes Subcutaneous Adipose Hyperplasia in Facial Port‐Wine Stains by Controlling HDAC1 Degradation and Stability Through Two Distinct Proteolytic Pathways

Advanced Science, EarlyView.
In PWS‐ASPCs, FOSL1 drives the expression of SPSB1. SPSB1, as part of the ESC complex, further binds to HDAC1 and promotes K29‐linked and K48‐linked polyubiquitination of HDAC1. These modifications facilitate the degradation of HDAC1 through the ALP and UPS pathways, respectively.
Hongrui Chen, Bin Sun, Yajing Qiu, Chen Hua, Xiaoxi Lin, Wei Gao   +5 more
wiley   +1 more source

A mouse model for fucosidosis recapitulates storage pathology and neurological features of the milder form of the human disease

Disease Models & Mechanisms, 2016
Fucosidosis is a rare lysosomal storage disorder caused by the inherited deficiency of the lysosomal hydrolase α-L-fucosidase, which leads to an impaired degradation of fucosylated glycoconjugates.
Heike Wolf, Markus Damme, Stijn Stroobants, Rudi D'Hooge, Hans Christian Beck, Irm Hermans-Borgmeyer, Renate Lüllmann-Rauch, Thomas Dierks, Torben Lübke   +8 more
doaj   +1 more source

Lysosomal Lipid Storage Disease from the Perspective of General Pediatricians

, 2016
Lizozomal lipid depo hastalıkları, lipid moleküllerinin katabolizmasında görevli enzimlerin eksikliği veya hücre içi taşınma kusurları sonucu meydana gelmektedir.
Ucar, Sema Kalkan, Beyhan Özkaya, Canda, Ebru Erbas, Sema Uçar Kalkan, Ozkaya, Beyhan, Ebru Canda Erbaş   +5 more
core   +1 more source

Perinatal Gene Transfer to the Liver [PDF]

, 2011
The liver acts as a host to many functions hence raising the possibility that any one may be compromised by a single gene defect. Inherited or de novo mutations in these genes may result in relatively mild diseases or be so devastating that death within
Buckley, SM, Ward, N.J., Buckley, S.M.K., Ward, NJ, Chan, J.K.Y., Waddington, S.N., Rahim, A.A., Waddington, SN, Rahim, AA, Chan, JK, Mckay, T.R., Howe, S.J., Howe, SJ, McKay, TR, Natalie J. Ward, Ahad A. Rahim, Steven J. Howe, Jerry K.Y.Chan, Suzanne M.K. Buckley, Simon N. Waddington, Tristan R. McKay   +20 more
core   +1 more source

Human Dental Pulp Stem Cell Secretome Restores Ischemic Stroke–Impaired Motor and Cognitive Functions by Reprogramming Redox and Inflammatory Signaling

Advanced Science, EarlyView.
Human dental pulp stem cell secretome emerges as a cell‐free therapeutic strategy for ischemic stroke by reprogramming redox and inflammatory signaling. hDPSC secretome‐derived antioxidant and immunomodulatory factors suppress the TLR4–NOX–ROS–NF‐κB axis, reduce microglial inflammatory responses and apoptosis, and promote neurogenesis, angiogenesis ...
Kyung‐Joo Seong, Sehoon Park, Sun‐Woong Bae, Daseul Kim, Jin Ho Lee, Won‐Seok Choi, Zee‐Yong Park, Ji‐Yeon Jung, Won‐Jae Kim   +8 more
wiley   +1 more source

Insulin-like growth factors in lysosomal storage disease.

, 1992
Recent data indicate that insulin-like growth factor II (IGF II) and lysosomal enzymes bind to a common receptor. We measured serum IGF I and II levels in 16 patients with various lysosomal storage disorders.
Froesch ER, Jaeggi-Groisman SE, Christomanou H, Kessler U, Kiess W   +4 more
core   +1 more source

Lysosomal storage diseases in non-immune hydrops fetalis pregnancies. [PDF]

, 2006
Contains fulltext : 50653.pdf (Publisher’s version ) (Open Access)BACKGROUND: At least 20 inborn errors of metabolism may cause hydrops fetalis. Most of these are lysosomal storage diseases. The study proposes a diagnostic flowchart for
Pim M.W. Janssens, Emilia K. Bijlsma, Janssens, Pim M.W., Smits, Arie P.T., Smits, Arie P. T., Janssens, P.M.W., van den Berg, Paul P., van den Berg, Catharina J.M.G., de Groot, Akosua N. J. A., Angelique J.A. Kooper, Catharina J.M.G. van den Berg, Berg, P.P. van den, Bijlsma, E.K., Liebrand-van Sambeek, Maria L.F., Arie P.T. Smits, Wevers, R.A., Kooper, Angelique J. A., Akosua N.J.A. de Groot, Smits, A.P.T., Janssens, Pim M. W., Maria L.F. Liebrand-van Sambeek, Kooper, A.J.A., van den Berg, Catharina J. M. G., Tan-Sindhunata, G.B., Ron A. Wevers, Gita B. Tan-Sindhunata, Berg, C.J.M.G. van den, Liebrand-van Sambeek, M.L.F., Bijlsma, Emilia K., Groot, A.N.J.A. de, Wevers, Ron A., Sambeek, Maria L. F. Liebrand-van, Paul P. van den Berg, Kooper, Angelique J.A., de Groot, Akosua N.J.A., Tan-Sindhunata, Gita B.   +35 more
core   +1 more source