Results 61 to 70 of about 1,083 (177)

The Influence of Schwann Cell Metabolism and Dysfunction on Axon Maintenance

Glia, Volume 73, Issue 12, Page 2338-2352, December 2025.
Main Points Sensory neurons depend on Schwann cells for survival. Schwann cells provide energy for axons dunng rapid firing or after injury. Dysregulated metabolism in Schwann cells can lead to the production of neurotoxic and axon degeneration. ABSTRACT Schwann cells are the glial cells in the peripheral nervous system responsible for the production ...
Rose Follis, Vishwanath V. Prabhu, Bruce D. Carter   +2 more
wiley   +1 more source

Characterization of Drosophila Saposin-related mutants as a model for lysosomal sphingolipid storage diseases

Disease Models & Mechanisms, 2017
Sphingolipidoses are inherited diseases belonging to the class of lysosomal storage diseases (LSDs), which are characterized by the accumulation of indigestible material in the lysosome caused by specific defects in the lysosomal degradation machinery ...
Julia Sellin, Heike Schulze, Marie Paradis, Dominic Gosejacob, Cyrus Papan, Andrej Shevchenko, Olympia Ekaterina Psathaki, Achim Paululat, Melanie Thielisch, Konrad Sandhoff, Michael Hoch   +10 more
doaj   +1 more source

ACE‐mediated Glycosylation Stabilizes PSAP To Promote GPR37‐dependent Macrophage‐Nucleus Pulposus Cells Crosstalk and TGFβ Signaling in Alleviating Intervertebral Disc Degeneration

Advanced Science, Volume 12, Issue 43, November 20, 2025.
ACE can modulate the CBL‐mediated K48 ubiquitination degradation of PSAP by altering its glycosylation levels in NP cells. As a result, NP cells secrete PSAP, which interacts with GPR37 on macrophage surfaces, facilitating their polarization toward the M2 phenotype. These M2 macrophages subsequently secrete TGFβ, which exerts feedback effects on the NP
Youfeng Guo, Feng Wang, Bijun Wang, Yu Zhou, Chao Wang, Tao Hu, Desheng Wu   +6 more
wiley   +1 more source

Structural similarities reveal an expansive conotoxin family with a two‐finger toxin fold

Protein Science, Volume 34, Issue 11, November 2025.
Abstract Venomous animals have evolved a diverse repertoire of toxins with considerable pharmaceutical potential. The rapid evolution of peptide toxins, such as the conotoxins produced by venomous marine cone snails, often complicates efforts to infer their evolutionary relationships based solely on sequence information.
Muhammad Saad Khilji, Celeste M. Hackney, Thomas L. Koch, Arik J. Hone, Aymeric Rogalski, Maren Watkins, Jortan Tun, J. Michael McIntosh, Baldomero Olivera, Helena Safavi‐Hemami, Kaare Teilum, Lars Ellgaard   +11 more
wiley   +1 more source

Generation and Auditory Phenotypic Characterization of Prps1 p.Ala87Thr Mouse Knock‐In Model for Human DFNX1 Deafness

Clinical Genetics, Volume 108, Issue 5, Page 566-575, November 2025.
Our mouse model with a c.259G>A transition in PRPS1 showed a significant decrease in the number of hair cells and SGN counts at 48 weeks of age and a reduction in Prps1 enzymatic activity in the KI mouse. This model will serve as a valuable tool for developing therapeutic strategies.
Denise Yan, M'hamed Grati, Rahul Mittal, Yi‐Zhou Quan, Wan Du, Zheng‐Yi Chen, Xue Zhong Liu   +6 more
wiley   +1 more source

Immunolocalization of the saposin-like insert of plant aspartic proteinases exhibiting saposin C activity. Expression in young flower tissues and in barley seeds

, 2005
The plant- specific insert ( PSI) of cypro11 gene- encoding cyprosin, an aspartic proteinase from Cynara cardunculus, has been cloned by polymerase chain reaction ( PCR) into a bacterial expression vector.
Marttila, S, Al-Karadaghi, Salam, Picaud, S, Hiraiwa, M, Brodelius, M, Brodelius, P E   +5 more
core   +1 more source

Host Transcriptomics Reveal Reduction in Defence‐Reproduction Trade‐Offs During Coinfection

Molecular Ecology, Volume 34, Issue 21, November 2025.
ABSTRACT During infection, hosts may shift resources away from reproduction towards immune defence. It is unclear to what degree these costly trade‐offs can be alleviated during protective coinfections, whereby antagonism between parasites reduces disease severity. We used transcriptomics to assess the extent to which host gene expression reflected the
Ian Will, Emily J. Stevens, Kayla C. King, Kieran A. Bates   +3 more
wiley   +1 more source

STRUCTURAL-ANALYSIS OF SAPOSIN-C AND SAPOSIN-B - COMPLETE LOCALIZATION OF DISULFIDE BRIDGES

, 1995
Saposins A, B, C, and D are a group of homologous glycoproteins derived from a single precursor, prosaposin, and apparently involved in the stimulation of the enzymatic degradation of sphingolipids in lysosomes. All saposins have six cysteine residues at
A. Amoresano, R. Ciaffoni, F. Zappacosta, R. Salvioli, R. Siciliano, P. Pucci, A. M. Vaccaro, A. Barca, M. Tatti, MARAS, Bruno   +9 more
core   +1 more source

Management of Gaucher Disease Type 1 in a Resource‐Limited Setting: A Pediatric Case Study

Clinical Case Reports, Volume 13, Issue 10, October 2025.
ABSTRACT This case report depicts the management of an 8‐year‐old male with Gaucher Disease Type 1, manifesting as massive splenomegaly, anemia, and skeletal involvement in a resource‐constrained environment. Treated with splenectomy due to the absence of enzyme replacement therapy, it underscores the necessity for enhanced therapeutic access and ...
Bipesh Kumar Shah, Diwakar Koirala, Bivek Mishra, Ramesh Sapkota, Manjil Basnet   +4 more
wiley   +1 more source

Stimulation of Acid Ceramidase Activity by Saposin D

Archives of Biochemistry and Biophysics, 1994
Ceramide is ubiquitously present in plasma membranes and plays a pivotal role in metabolism of sphingolipids. In addition, ceramide and its hydrolytic product, sphingosine, may have important roles as second messengers for cell function and survival. Ceramide is hydrolyzed by both acid and alkaline ceramidase. In this investigation, saposin D was found
N, Azuma, J S, O'Brien, H W, Moser, Y, Kishimoto   +3 more
openaire   +2 more sources