PMID- 18266119
OWN - NLM
STAT- MEDLINE
DCOM- 20080603
LR  - 20211020
IS  - 1350-6129 (Print)
IS  - 1744-2818 (Electronic)
IS  - 1350-6129 (Linking)
VI  - 15
IP  - 1
DP  - 2008 Mar
TI  - Biochemical and aggregation analysis of Bence Jones proteins from different light 
      chain diseases.
PG  - 29-39
LID - 10.1080/13506120701815324 [doi]
AB  - Deposition of immunoglobulin light chains is a result of clonal proliferation of 
      monoclonal plasma cells that secrete free immunoglobulin light chains, also 
      called Bence Jones proteins (BJP). These BJP are present in circulation in large 
      amounts and excreted in urine in various light chain diseases such as light chain 
      amyloidosis (AL), light chain deposition disease (LCDD) and multiple myeloma 
      (MM). BJP from patients with AL, LCDD and MM were purified from their urine and 
      studies were performed to determine their secondary structure, thermodynamic 
      stability and aggregate formation kinetics. Our results show that LCDD and MM 
      proteins have the lowest free energy of folding while all proteins show similar 
      melting temperatures. Incubation of the BJP at their melting temperature produced 
      morphologically different aggregates: amyloid fibrils from the AL proteins, 
      amorphous aggregates from the LCDD proteins and large spherical species from the 
      MM proteins. The aggregates formed under in vitro conditions suggested that the 
      various proteins derived from patients with different light chain diseases might 
      follow different aggregation pathways.
FAU - Sikkink, Laura A
AU  - Sikkink LA
AD  - Department of Biochemistry and Molecular Biology, Mayo Clinic College of 
      Medicine, Rochester, Minnesota 55905, USA.
FAU - Ramirez-Alvarado, Marina
AU  - Ramirez-Alvarado M
LA  - eng
GR  - R01 GM071514/GM/NIGMS NIH HHS/United States
GR  - GM071514/GM/NIGMS NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, Non-U.S. Gov't
PL  - England
TA  - Amyloid
JT  - Amyloid : the international journal of experimental and clinical investigation : 
      the official journal of the International Society of Amyloidosis
JID - 9433802
RN  - 0 (Immunoglobulin Light Chains)
RN  - 9006-99-9 (Bence Jones Protein)
SB  - IM
MH  - *Amyloidosis/metabolism
MH  - Bence Jones Protein/*chemistry/isolation & purification/metabolism
MH  - Humans
MH  - Immunoglobulin Light Chains
MH  - *Multiple Myeloma/metabolism
MH  - *Protein Folding
MH  - Protein Structure, Secondary
MH  - Thermodynamics
PMC - PMC2556190
EDAT- 2008/02/13 09:00
MHDA- 2008/06/05 09:00
PMCR- 2008/09/29
CRDT- 2008/02/13 09:00
PHST- 2008/02/13 09:00 [pubmed]
PHST- 2008/06/05 09:00 [medline]
PHST- 2008/02/13 09:00 [entrez]
PHST- 2008/09/29 00:00 [pmc-release]
AID - 790527898 [pii]
AID - 10.1080/13506120701815324 [doi]
PST - ppublish
SO  - Amyloid. 2008 Mar;15(1):29-39. doi: 10.1080/13506120701815324.