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Logo of jcinvestThe Journal of Clinical Investigation
J Clin Invest. 1985 December; 76(6): 2425–2429.
PMCID: PMC424400

Beta-2 microglobulin is an amyloidogenic protein in man.


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.

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Selected References

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  • Glenner GG. Amyloid deposits and amyloidosis. The beta-fibrilloses (first of two parts). N Engl J Med. 1980 Jun 5;302(23):1283–1292. [PubMed]
  • Saraiva MJ, Birken S, Costa PP, Goodman DS. Amyloid fibril protein in familial amyloidotic polyneuropathy, Portuguese type. Definition of molecular abnormality in transthyretin (prealbumin). J Clin Invest. 1984 Jul;74(1):104–119. [PMC free article] [PubMed]
  • Cohen DH, Feiner H, Jensson O, Frangione B. Amyloid fibril in hereditary cerebral hemorrhage with amyloidosis (HCHWA) is related to the gastroentero-pancreatic neuroendocrine protein, gamma trace. J Exp Med. 1983 Aug 1;158(2):623–628. [PMC free article] [PubMed]
  • Masters CL, Simms G, Weinman NA, Multhaup G, McDonald BL, Beyreuther K. Amyloid plaque core protein in Alzheimer disease and Down syndrome. Proc Natl Acad Sci U S A. 1985 Jun;82(12):4245–4249. [PubMed]
  • Lesk AM, Chothia C. Evolution of proteins formed by beta-sheets. II. The core of the immunoglobulin domains. J Mol Biol. 1982 Sep 15;160(2):325–342. [PubMed]
  • Blake CC, Geisow MJ, Oatley SJ, Rérat B, Rérat C. Structure of prealbumin: secondary, tertiary and quaternary interactions determined by Fourier refinement at 1.8 A. J Mol Biol. 1978 May 25;121(3):339–356. [PubMed]
  • Glenner GG, Wong CW. Alzheimer's disease: initial report of the purification and characterization of a novel cerebrovascular amyloid protein. Biochem Biophys Res Commun. 1984 May 16;120(3):885–890. [PubMed]
  • Clanet M, Mansat M, Durroux R, Testut MF, Guiraud B, Rascol A, Conte J. Syndrome du canal carpien, ténosynovite amyloíde et hémodialyse périodique. Rev Neurol (Paris) 1981;137(10):613–624. [PubMed]
  • Kachel HG, Altmeyer P, Baldamus CA, Koch KM. Deposition of an amyloid-like substance as a possible complication of regular dialysis treatment. Contrib Nephrol. 1983;36:127–132. [PubMed]
  • Laemmli UK. Cleavage of structural proteins during the assembly of the head of bacteriophage T4. Nature. 1970 Aug 15;227(5259):680–685. [PubMed]
  • Frangione B, Rosenwasser E, Penefsky HS, Pullman ME. Amino acid sequence of the protein inhibitor of mitochondrial adenosine triphosphatase. Proc Natl Acad Sci U S A. 1981 Dec;78(12):7403–7407. [PubMed]
  • Gorevic PD, Cleveland AB, Franklin EC. The biologic significance of amyloid. Ann N Y Acad Sci. 1982;389:380–394. [PubMed]
  • Karlsson FA. Physical-chemical properties of beta 2-microglobulin. Immunochemistry. 1974 Mar;11(3):111–114. [PubMed]
  • Lancet D, Parham P, Strominger JL. Heavy chain of HLA-A and HLA-B antigens is conformationally labile: a possible role for beta 2-microglobulin. Proc Natl Acad Sci U S A. 1979 Aug;76(8):3844–3848. [PubMed]
  • Becker JW, Reeke GN., Jr Three-dimensional structure of beta 2-microglobulin. Proc Natl Acad Sci U S A. 1985 Jun;82(12):4225–4229. [PubMed]
  • Michaelson J, Rothenberg E, Boyse EA. Genetic polymorphism of murine beta 2-microglobulin detected biochemically. Immunogenetics. 1980 Jul;11(1):93–95. [PubMed]
  • Smith KK, Parham P, Ma NS. Two distinct forms of beta 2-microglobulin in different races of owl monkey (Aotus trivirgatus). Immunogenetics. 1984;20(4):459–464. [PubMed]
  • Cunningham BA, Wang JL, Berggård I, Peterson PA. The complete amino acid sequence of beta 2-microglobulin. Biochemistry. 1973 Nov 20;12(24):4811–4822. [PubMed]
  • Hall PW, Ricanati ES, Vacca CV. Characterization of human beta2-microglobulin by isoelectric focusing. Clin Chim Acta. 1977 May 16;77(1):37–42. [PubMed]
  • Dwulet FE, Benson MD. Primary structure of an amyloid prealbumin and its plasma precursor in a heredofamilial polyneuropathy of Swedish origin. Proc Natl Acad Sci U S A. 1984 Feb;81(3):694–698. [PubMed]
  • Lian JB, Skinner M, Benson MD, Cohen AS. Fractionation of primary amyloid fibrils. Characterization and chemical interaction of the subunits. Biochim Biophys Acta. 1977 Mar 28;491(1):167–176. [PubMed]
  • Talal N, Grey HM, Zvaifler N, Michalski JP, Daniels TE. Elevated salivary and synovial fluid beta2-microglobulin in Sjogren's syndrome and rheumatoid arthritis. Science. 1975 Mar 28;187(4182):1196–1198. [PubMed]
  • Child JA, Kushwaha MR. Serum beta 2-microglobulin in lymphoproliferative and myeloproliferative diseases. Hematol Oncol. 1984 Oct-Dec;2(4):391–401. [PubMed]
  • Randall RE, Williamson WC, Jr, Mullinax F, Tung MY, Still WJ. Manifestations of systemic light chain deposition. Am J Med. 1976 Feb;60(2):293–299. [PubMed]
  • Orfila C, Rakotoarivony J, Durand D, Segonds-Modesto A, Suc JM. Immunofluorescence and ultrastructural localization of beta 2-microglobulin in human renal allografts. Histochemistry. 1984;81(6):533–541. [PubMed]
  • Gejyo F, Yamada T, Odani S, Nakagawa Y, Arakawa M, Kunitomo T, Kataoka H, Suzuki M, Hirasawa Y, Shirahama T, et al. A new form of amyloid protein associated with chronic hemodialysis was identified as beta 2-microglobulin. Biochem Biophys Res Commun. 1985 Jun 28;129(3):701–706. [PubMed]

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