Involvement of Macrophages in the Pathogenesis of Familial Amyloid Polyneuropathy and Efficacy of Human iPS Cell-Derived Macrophages in Its Treatment

PloS One
Genki SuenagaYukio Ando

Abstract

We hypothesized that tissue-resident macrophages in familial amyloid polyneuropathy (FAP) patients will exhibit qualitative or quantitative abnormalities, that may accelerate transthyretin (TTR)-derived amyloid deposition. To evaluate this, we examined the number and subset of tissue-resident macrophages in heart tissue from amyloid-deposited FAP and control patients. In both FAP and control patients, tissue-resident macrophages in heart tissue were all Iba+/CD163+/CD206+ macrophages. However, the number of macrophages was significantly decreased in FAP patients compared with control patients. Furthermore, the proportion of intracellular TTR in CD14+ monocytes was reduced in peripheral blood compared with healthy donors. Based on these results, we next examined degradation and endocytosis of TTR in human induced pluripotent stem (iPS) cell-derived myeloid lineage cells (MLs), which function like macrophages. iPS-MLs express CD163 and CD206, and belong to the inhibitory macrophage category. In addition, iPS-MLs degrade both native and aggregated TTR in a cell-dependent manner in vitro. Further, iPS-MLs endocytose aggregated, and especially polymerized, TTR. These results suggest that decreased tissue-localized macrophages disrup...Continue Reading

References

May 23, 1986·Neuroscience Letters·P W Dickson, G Schreiber
Sep 1, 1968·The Journal of Clinical Investigation·M KanaiD S Goodman
Jun 20, 1998·Liver Transplantation and Surgery : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·A W HemmingG A Levy
Jun 5, 2001·Current Opinion in Pediatrics·A S Batra, A B Lewis
Jan 4, 2003·Nature Reviews. Immunology·Siamon Gordon
Aug 22, 2003·Journal of Internal Medicine·O B SuhrP J Ranløv
Aug 1, 1958·Endocrinology·S H INGBAR
Dec 20, 2003·Nature·Fred E Cohen, Jeffery W Kelly
Oct 14, 2004·Annals of Medicine·Søren K Moestrup, Holger J Møller
Sep 1, 2005·Experimental Eye Research·Takahiro KawajiHidenobu Tanihara
Oct 4, 2006·The Journal of Immunology : Official Journal of the American Association of Immunologists·Kei OnoYoshio Kuroki
Jun 8, 2007·Muscle & Nerve·Merrill D Benson, John C Kincaid
Apr 3, 2008·Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·Ayako TsuchiyaShu-ichi Ikeda
Sep 5, 2008·The Journal of Immunology : Official Journal of the American Association of Immunologists·Marie BenoitJean-Louis Mege
Oct 28, 2008·Clinical Immunology : the Official Journal of the Clinical Immunology Society·Marcin MoniuszkoMilena Dabrowska
Mar 12, 2010·American Journal of Physiology. Regulatory, Integrative and Comparative Physiology·James G Tidball, S Armando Villalta
Jul 16, 2010·Blood·Loems Ziegler-HeitbrockManfred B Lutz
Aug 7, 2010·Proceedings of the Japan Academy. Series B, Physical and Biological Sciences·Shukuro Araki, Yukio Ando
Nov 1, 2011·Folia Histochemica Et Cytobiologica·Krzysztof KowalOtylia Kowal-Bielecka
Apr 5, 2012·Current Medicinal Chemistry·Y Ando, M Ueda
Apr 11, 2012·Journal of Innate Immunity·Elizabeth E Gray, Jason G Cyster
Sep 6, 2012·Circulation·Frederick L Ruberg, John L Berk
Sep 11, 2012·Cancer Science·Yoshihiro KomoharaMotohiro Takeya
Nov 22, 2012·Muscle & Nerve·Merrill D Benson
Apr 27, 2013·Nature·Thomas A WynnJeffrey W Pollard
Jul 3, 2013·Laboratory Investigation; a Journal of Technical Methods and Pathology·Yohei MisumiMaria J Saraiva
Jun 12, 2014·Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis·Nádia Pereira GonçalvesMaria João Saraiva
Nov 5, 2014·Frontiers in Immunology·Paola Italiani, Diana Boraschi
Dec 30, 2014·Frontiers in Immunology·Charles D MillsMarkus Munder
Jun 30, 2015·The American Journal of Pathology·Amitava DasSashwati Roy
Nov 28, 2015·Annals of Medicine·Philip N HawkinsOle B Suhr
Dec 15, 2015·Neurology and Therapy·Márcia Waddington Cruz, Merril D Benson
Jan 15, 2016·Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis·Kosuke OkumuraYukio Ando

❮ Previous
Next ❯

Citations

Jul 26, 2019·Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis·João MoreiraMaria João Saraiva
Jun 7, 2017·Journal of Neuroinflammation·Nádia Pereira GonçalvesMaria João Saraiva
Jul 2, 2020·Cells·Mohammed M H AsiriJo W M Höppener
Jun 8, 2021·Frontiers in Immunology·João MoreiraMaria João Saraiva

❮ Previous
Next ❯

Methods Mentioned

BETA
flow-cytometry
enzyme-linked immunosorbent assay
FACS
ELISA
Assay
flow cytometry

Related Concepts

Related Feeds

Adenomatous Polyposis Coli

Adenomatous polyposis coli is a protein encoded by the APC gene and acts as a tumor suppressor. Discover the latest research on adenomatous polyposis coli here.

Birth Defects

Birth defects encompass structural and functional alterations that occur during embryonic or fetal development and are present since birth. The cause may be genetic, environmental or unknown and can result in physical and/or mental impairment. Here is the latest research on birth defects.

Amyloidogenesis-associated Polyneuropathy

Amyloid deposits around nerves can damage and disrupt smooth information processing between the brain and organs. Here is the latest research on amyloidosis-associated polyneuropathies.

Related Papers

AJR. American Journal of Roentgenology
Gianluca Di BellaMichele Gaeta
Clinica Chimica Acta; International Journal of Clinical Chemistry
Noboru HanyuHiroya Hidaka
Laboratory Investigation; a Journal of Technical Methods and Pathology
Yohei MisumiM J Saraiva
© 2021 Meta ULC. All rights reserved