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Purification and Aggregation of the Amyloid Precursor Protein Intracellular Domain
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Influence of a Single Point Mutation in the Constant Domain of the Bence-Jones Protein bif on Its Aggregation

M A Timchenko1, A A Timchenko

  • 1Institute of Theoretical and Experimental Biophysics, Russian Academy of Sciences, Pushchino, Moscow Region, 142290, Russia. maria_timchenko@mail.ru.

Biochemistry. Biokhimiia
|April 6, 2018
PubMed

Insights

The constant domain of Bence-Jones proteins, not just variable domains, can drive amyloid formation in multiple myeloma kidney disease. A specific mutation (N177S) alters this aggregation process, impacting kidney health.

Area of Science:

  • Nephrology
  • Molecular Biology
  • Immunology

Background:

  • Multiple myeloma nephropathy involves kidney damage from Bence-Jones protein aggregates.
  • Amyloid deposit formation mechanisms in the kidneys are not fully understood.
  • Previous research focused on variable domains of light chains causing amyloidogenesis.

Purpose of the Study:

  • To investigate the role of the constant domain in Bence-Jones protein aggregation.
  • To analyze the impact of a specific N177S mutation in the constant domain on amyloid formation.
  • To compare the aggregation behavior of wild-type Bence-Jones protein BIF with its N177S mutant.

Main Methods:

  • Isolation of recombinant Bence-Jones protein BIF and its N177S mutant.
  • Atomic force microscopy to analyze aggregate morphology.
  • Incubation of proteins under simulated physiological conditions (pH, reducing agents).

Main Results:

  • Wild-type BIF formed fibrils across a range of pH conditions (7.2, 6.5, 10.1).
  • The N177S mutant formed fibrils only at alkaline pH (10.1).
  • Both proteins formed branched structures upon refolding with a reducing agent (dithiothreitol).

Conclusions:

  • The constant domain of Bence-Jones proteins plays a critical role in amyloidogenesis.
  • The N177S mutation significantly alters the aggregation properties of Bence-Jones proteins.
  • pH and reducing conditions influence the type of aggregates formed, relevant to kidney pathology.

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