PMID- 33163710
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20201112
IS  - 2468-0249 (Electronic)
IS  - 2468-0249 (Linking)
VI  - 5
IP  - 11
DP  - 2020 Nov
TI  - Understanding Mesangial Pathobiology in AL-Amyloidosis and Monoclonal Ig Light 
      Chain Deposition Disease.
PG  - 1870-1893
LID - 10.1016/j.ekir.2020.07.013 [doi]
AB  - Patients with plasma cell dyscrasias produce free abnormal monoclonal Ig light 
      chains that circulate in the blood stream. Some of them, termed glomerulopathic 
      light chains, interact with the mesangial cells and trigger, in a manner 
      dependent of their structural and physicochemical properties, a sequence of 
      pathological events that results in either light chain-derived (AL) amyloidosis 
      (AL-Am) or light chain deposition disease (LCDD). The mesangial cells play a key 
      role in the pathogenesis of both diseases. The interaction with the pathogenic 
      light chain elicits specific cellular processes, which include apoptosis, 
      phenotype transformation, and secretion of extracellular matrix components and 
      metalloproteinases. Monoclonal light chains associated with AL-Am but not those 
      producing LCDD are avidly endocytosed by mesangial cells and delivered to the 
      mature lysosomal compartment where amyloid fibrils are formed. Light chains from 
      patients with LCDD exert their pathogenic signaling effect at the cell surface of 
      mesangial cells. These events are generic mesangial responses to a variety of 
      adverse stimuli, and they are similar to those characterizing other more frequent 
      glomerulopathies responsible for many cases of end-stage renal disease. The 
      pathophysiologic events that have been elucidated allow to propose future 
      therapeutic approaches aimed at preventing, stopping, ameliorating, or reversing 
      the adverse effects resulting from the interactions between glomerulopathic light 
      chains and mesangium.
CI  - (c) 2020 International Society of Nephrology. Published by Elsevier Inc.
FAU - Herrera, Guillermo A
AU  - Herrera GA
AD  - Department of Pathology, College of Medicine-University of South Alabama, Mobile, 
      Alabama, USA.
AD  - Department of Physiology and Cell Biology, College of Medicine-University of 
      South Alabama, Mobile, Alabama, USA.
FAU - Teng, Jiamin
AU  - Teng J
AD  - Department of Pathology, College of Medicine-University of South Alabama, Mobile, 
      Alabama, USA.
FAU - Turbat-Herrera, Elba A
AU  - Turbat-Herrera EA
AD  - Department of Pathology, College of Medicine-University of South Alabama, Mobile, 
      Alabama, USA.
AD  - Mitchell Cancer Institute-University of South Alabama, Mobile, Alabama, USA.
FAU - Zeng, Chun
AU  - Zeng C
AD  - Department of Pathology, College of Medicine-University of South Alabama, Mobile, 
      Alabama, USA.
FAU - Del Pozo-Yauner, Luis
AU  - Del Pozo-Yauner L
AD  - Department of Pathology, College of Medicine-University of South Alabama, Mobile, 
      Alabama, USA.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20200721
PL  - United States
TA  - Kidney Int Rep
JT  - Kidney international reports
JID - 101684752
PMC - PMC7609979
OTO - NOTNLM
OT  - AL-amyloidosis
OT  - kidney
OT  - kidney repair
OT  - light chain deposition disease
OT  - mesangium
OT  - monoclonal Ig deposition disease
OT  - monoclonal light chains
OT  - stem cells
EDAT- 2020/11/10 06:00
MHDA- 2020/11/10 06:01
PMCR- 2020/07/21
CRDT- 2020/11/09 05:34
PHST- 2020/05/20 00:00 [received]
PHST- 2020/07/06 00:00 [revised]
PHST- 2020/07/14 00:00 [accepted]
PHST- 2020/11/09 05:34 [entrez]
PHST- 2020/11/10 06:00 [pubmed]
PHST- 2020/11/10 06:01 [medline]
PHST- 2020/07/21 00:00 [pmc-release]
AID - S2468-0249(20)31378-4 [pii]
AID - 10.1016/j.ekir.2020.07.013 [doi]
PST - epublish
SO  - Kidney Int Rep. 2020 Jul 21;5(11):1870-1893. doi: 10.1016/j.ekir.2020.07.013. 
      eCollection 2020 Nov.