Light chain deposition disease (LCDD) is a multisystemic disorder seen in the setting of plasma cell dyscrasias. The histological characteristic of this disorder is the deposition of a homogeneous, granular, slightly eosinophilic and non-Congophilic material that shows immunostaining for monoclonal light chains (kappa or gamma), while in primary amyloidosis (AL) the proteinaceous substance is fibrillar and Congo red positive. In contrast with AL, the light chain in LCDD is usually of the kappa-type. Renal involvement, resulting in nephrotic syndrome, is usually the prominent feature of LCDD. Patients with this disease may also have heart, liver or other organ involvement, mimicking the picture of primary systemic amyloidosis. However, liver failure has rarely been described in patients with LCDD. A patient with myeloma-associated LCDD who developed rapidly progressive liver kappa light chain deposition with fatal outcome after undergoing the first cycle of vincristine/doxorubicin/dexamethasone chemotherapy is reported.
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http://dx.doi.org/10.1111/j.1600-0609.2005.00561.x | DOI Listing |
Angew Chem Int Ed Engl
January 2025
Southeast University, Institute of Advanced Materials and School of Chemistry and Chemical Engineering, Institute of Advanced Materials and School of Chemistry and Chemical Engineering, 211189, Nanjing, CHINA.
In nature, organisms adapt to environmental changes through training to learn new abilities, offering valuable insights for developing intelligent materials. However, replicating this adaptive learning in synthetic materials presents a significant challenge. This study introduces a feasible approach to train liquid crystal elastomers (LCEs) by integrating a mechanophore tetraarylsuccinonitrile (TASN) into their main chain, addressing the challenge of enabling synthetic materials to exchange substances with their environment.
View Article and Find Full Text PDFAnn Clin Transl Neurol
January 2025
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
Defining the CSF cytokine/chemokine and injury biomarker signature of glial fibrillary acidic protein (GFAP) autoimmunity can inform immunopathogenesis. CSF GFAP-IgG-positive samples (N = 98) were tested for 17 cytokines/chemokines, neurofilament light chain (NfL), and GFAP (ELLA, Bio-Techne). Controls included non-inflammatory (N = 42), AQP4-IgG-positive (N = 83), CNS infections (N = 13), and neurosarcoidosis (N = 32).
View Article and Find Full Text PDFPlant J
January 2025
Systems Biotechnology Group, Department Microbial Biotechnology, Helmholtz Centre for Environmental Research - UFZ, Leipzig, 04318, Germany.
Biophotovoltaics offers a promising low-carbon footprint approach to utilize solar energy. It aims to couple natural oxygenic photosynthetic electrons to an external electron sink. This lays the foundation for a potentially high light-to-energy efficiency of the Biophotovoltaic process.
View Article and Find Full Text PDFThe folded auto-inhibited state of kinesin-1 is stabilized by multiple weak interactions and binds weakly to microtubules. Here we investigate the extent to which homodimeric kinesin-1 lacking light chains is activated by the dynein activating adaptor BicD. We show that one or two kinesins can bind to the central region of BicD (CC2), a region distinct from that which binds dynein-dynactin (CC1) and cargo-adaptor proteins (CC3).
View Article and Find Full Text PDFCytoplasmic dynein is an essential microtubule motor protein that powers organelle transport and mitotic spindle assembly. Its activity depends on dynein-dynactin-cargo adaptor complexes, such as dynein-dynactin-BicD2 (DDB), which typically function with two dynein motors. We show that mechanical tension recruits a third dynein motor via an auxiliary BicD adaptor binding the light intermediate chain of the third dynein, stabilizing multi-dynein assemblies and enhancing force generation.
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