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Updated: Apr 19, 2026

Purification and Aggregation of the Amyloid Precursor Protein Intracellular Domain
10:08

Purification and Aggregation of the Amyloid Precursor Protein Intracellular Domain

Published on: August 28, 2012

12.4K

C-terminal unfolding of an amyloidogenic β2-microglobulin fragment: ΔN6β2-microglobulin

Yoshihiro Motomiya1, Yuichiro Higashimoto, Yoshinori Uji

  • 1Suiyukai Clinic , Kashihara, Nara , Japan .

Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis
|December 20, 2014
PubMed
Abstract

Insights

Researchers studied a fragment of beta2-microglobulin (β2m), called ΔN6β2m, and its role in dialysis-related amyloidosis. They found C-terminal unfolding in ΔN6β2m but did not detect the amyloidogenic intermediate β2m92-99 in hemodialysis patients.

Area of Science:

  • Biochemistry
  • Proteomics
  • Medical Diagnostics

Background:

  • Dialysis-related amyloidosis is linked to beta2-microglobulin (β2m) amyloid formation.
  • A specific fragment, ΔN6β2-microglobulin (ΔN6β2m), is amyloidogenic.
  • C-terminal unfolding of β2m is implicated in its amyloidogenicity.

Purpose of the Study:

  • Investigate the conformational characteristics of ΔN6β2m related to its amyloidogenicity.
  • Determine if C-terminal unfolding occurs in ΔN6β2m.
  • Measure the concentration of an amyloidogenic intermediate (β2m92-99) in hemodialysis patients' serum.

Main Methods:

  • Capillary electrophoretic analysis.
  • Surface plasmon resonance.
  • Enzyme-linked immunosorbent assay (ELISA).

Main Results:

  • Confirmed the normal core structure of ΔN6β2m.
  • Verified C-terminal region unfolding in ΔN6β2m using a specific antibody (mAb 92-99).
  • Developed an ELISA to measure β2m92-99, using ΔN6β2m as a standard.

Conclusions:

  • ΔN6β2m exhibits C-terminal unfolding, a key feature for amyloidogenicity.
  • Despite assay development, the specific amyloidogenic intermediate β2m92-99 was not detected in the serum of 10 hemodialysis patients.
  • Further research may be needed to understand the in vivo presence of this intermediate.
Keywords:
Amyloidogenicitycapillary electrophoresisdialysis-related amyloidosisenzyme-linked immunosorbent assayhemodialysismonoclonal antibody 92-99surface plasmon resonance

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