RT Book, Section
A1 Berk, John L.
A1 Sanchorawala, Vaishali
A2 Jameson, J. Larry
A2 Fauci, Anthony S.
A2 Kasper, Dennis L.
A2 Hauser, Stephen L.
A2 Longo, Dan L.
A2 Loscalzo, Joseph
SR Print(0)
ID 1156755079
T1 Amyloidosis
T2 Harrison's Principles of Internal Medicine, 20e
YR 2018
FD 2018
PB McGraw-Hill Education
PP New York, NY
SN 9781259644016
LK accesspharmacy.mhmedical.com/content.aspx?aid=1156755079
RD 2024/04/18
AB Amyloidosis  is  the  term  for  a  group  of  protein  misfolding  disorders  characterized  by  the  extracellular  deposition  of  insoluble  polymeric  protein  fibrils  in  tissues  and  organs.  A  robust  cellular  machinery  exists  to  chaperone  proteins  during  the  process  of  synthesis  and  secretion,  to  ensure  that  they  achieve  correct  tertiary  conformation  and  function,  and  to  eliminate  proteins  that  misfold.  However,  genetic  mutation,  incorrect  processing,  and  other  factors  may  favor  misfolding,  with  consequent  loss  of  normal  protein  function  and  intracellular  or  extracellular  aggregation.  Many  diseases,  ranging  from  cystic  fibrosis  to  Alzheimer’s  disease,  are  now  known  to  involve  protein  misfolding.  In  the  amyloidoses,  the  aggregates  are  typically  extracellular,  and  the  misfolded  protein  subunits  assume  a  common  antiparallel,  β-pleated  sheet–rich  structural  conformation  that  leads  to  the  formation  of  higher-order  oligomers  and  then  fibrils  with  unique  staining  properties.  The  term  amyloid  was  coined  around  1854  by  the  pathologist  Rudolf  Virchow,  who  thought  that  these  deposits  resembled  starch  (Latin  amylum)  under  the  microscope.