Introduction: Cystinuria, a rare autosomal recessive disease characterized by a defect in cystine renal reabsorption, can often determine complex cystine renal calculi, leading to important complications such as urinary obstruction, urinary infections, and impaired kidney function. Complex kidney stones can have a difficult management and can be very arduous to treat.
Case Description: We present the case of a 20-year-old Jeowah's witness woman with complex cystine renal stones treated with combined robot-assisted laparoscopic pyelolithotomy and intraoperative renoscopy.
Conclusions: The combination of robot-assisted laparoscopic pyelolithotomy and intraoperative renoscopy can be useful to achieve an immediate high stone clearance rate also in complex renal stones. This combined technique could be indicated to minimize intraoperative bleeding. Moreover, it can also be used in pediatric cases or when there is no ureteral compliance. However, this strategy can be performed only in hospital referral centers by expert surgeons.
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http://dx.doi.org/10.5301/uro.5000215 | DOI Listing |
Mol Divers
January 2025
Department of Microbiology, Maharshi Dayanand University, Rohtak, Haryana, 124001, India.
Cyclotides are a class of plant-derived cyclic peptides having a distinctive structure with a cyclic cystine knot (CCK) motif. They are stable molecules that naturally play a role in plant defense. Till date, more than 750 cyclotides have been reported among diverse plant taxa belonging to Cucurbitaceae, Violaceae, Rubiaceae, Solanaceae, and Fabaceae.
View Article and Find Full Text PDFbioRxiv
January 2025
Department of Biochemistry and Molecular Biophysics, Columbia University, New York, NY 10032.
The glycoprotein hormones of humans, produced in the pituitary and acting through receptors in the gonads to support reproduction and in the thyroid gland for metabolism, have co-evolved from invertebrate counterparts. These hormones are heterodimeric cystine-knot proteins; and their receptors bind the cognate hormone at an extracellular domain and transmit the signal of this binding through a transmembrane domain that interacts with a heterotrimeric G protein. Structures determined for the human receptors as isolated for cryogenic electron microscopy (cryo-EM) are all monomeric despite compelling evidence for their functioning as dimers.
View Article and Find Full Text PDFWhile it has been appreciated for decades that lysosomes can import cysteine, its for organismal physiology is unclear. Recently, the MFSD12 transmembrane protein was shown to be necessary to import cysteine into lysosomes (and melanosomes), enabling the study of these processes using genetic tools. Here, we find that mice lacking die between embryonic days 10.
View Article and Find Full Text PDFElife
January 2025
Departments of Molecular & Cellular Physiology and Structural Biology, Stanford University School of Medicine, Stanford, United States.
Wnt/β-catenin signaling directs animal development and tissue renewal in a tightly controlled, cell- and tissue-specific manner. In the mammalian central nervous system, the atypical ligand Norrin controls angiogenesis and maintenance of the blood-brain barrier and blood-retina barrier through the Wnt/β-catenin pathway. Like Wnt, Norrin activates signaling by binding and heterodimerizing the receptors Frizzled (Fzd) and low-density lipoprotein receptor-related protein 5 or 6 (LRP5/6), leading to membrane recruitment of the intracellular transducer Dishevelled (Dvl) and ultimately stabilizing the transcriptional coactivator β-catenin.
View Article and Find Full Text PDFJ Colloid Interface Sci
December 2024
State Key Laboratory of Heavy Oil Processing and Centre for Bioengineering and Biotechnology, College of Chemical Engineering, China University of Petroleum (East), 66 Changjiang West Road, Qingdao 266580, China.
Efficient intracellular delivery of native proteins remains a big challenge, which greatly hinders the development of protein therapy. Here, we report a generalizable peptide vector that can encapsulate and deliver various proteins to achieve efficient intracellular biocatalysis. The peptide was rationally designed to be cationic amphiphilic peptide that consist of four functional fragments, that is, a hydrophobic domain to promote molecular assembly, an enzyme-cleavable fragment to introduce stimuli-responsibility, several cationic arginine (Arg) residues to enhance cell interaction and transmembrane efficiency, and the cystine (Cys) residues with redox sensitivity to adjust the stability of the peptide/protein complexes as needed.
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