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PMID: 3908488 Published · ppublish English Case Reports Journal Article Research Support, U.S. Gov't, P.H.S.

Beta-2 microglobulin is an amyloidogenic protein in man.

The Journal of clinical investigation ·Vol. 76 ·No. 6 ·1985-12-00 ·Pages 2425-9

Gorevic PD, Casey TT, Stone WJ, DiRaimondo CR, Prelli FC, Frangione B

Abstract

Curvilinear fibrils with the tinctorial properties of amyloid were isolated from a patient with bone and joint involvement complicating chronic dialysis for renal disease. Subunit fractions of 24,000 and 12,000 mol wt were identified after gel filtration under dissociating conditions, the latter containing a significant amount of a dimer of the former. This was confirmed by Edman degradation of each fraction, which yielded the amino terminal sequence of normal human beta-2 microglobulin (B2M) to residues 20 and 30, respectively. The size of the subunit protein (12,000 mol wt) and the amino acid composition make it likely that intact B2M is a major constituent of the fibrils. B2M is thus another example of a low molecular weight serum protein, with a prominent beta-pleated sheet structure, that may adopt the fibrillar configuration of amyloid in certain pathologic states.

MeSH Terms
Aged Amino Acid Sequence Amyloid/metabolism Amyloidosis/complications,metabolism Bone Diseases/complications,metabolism Humans Kidney Failure, Chronic/complications,metabolism Male beta 2-Microglobulin/metabolism
Chemicals
Amyloid beta 2-Microglobulin
Authors & Affiliations
6 authors, click to expand affiliations / ORCID
Gorevic P D
Casey T T
Stone W J
DiRaimondo C R
Prelli F C
Frangione B
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Article Info
Journal
The Journal of clinical investigation
Abbr.
J Clin Invest
ISSN
0021-9738
DOI
10.1172/JCI112257
Published
1985-12-00
Pages
2425-9
Language
English
Region
United States
NLM ID
7802877
PMCID
PMC424400
Subset
IM
Grants
NIADDK NIH HHS · AM 01431 · United States
NIADDK NIH HHS · AM 02594 · United States
NIGMS NIH HHS · GM 31866 · United States
External Links
PubMed source DOI: 10.1172/JCI112257 PMC Full Text View journal details
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