Related Experiment Video

Updated: Aug 21, 2026

Protein Misfolding Cyclic Amplification of Prions
10:12

Protein Misfolding Cyclic Amplification of Prions

Published on: November 7, 2012

Doppel: the prion's double

Ewa Golańiska1, Marcin Flirski, Paweł P Liberski

  • 1Department of Molecular Pathology and Neuropathology, Medical University of Lódź, Lódź, Poland. golanska@wp.pl

Folia Neuropathologica
|September 29, 2004
PubMed

Insights

Doppel (Dpl) protein, similar to PrP, has distinct functions and is not involved in prion propagation. Research on its role in neurodegenerative diseases like CJD remains inconclusive due to contradictory findings.

Area of Science:

  • Biochemistry
  • Genetics
  • Neuroscience

Background:

  • Doppel (Dpl) is a PrP-like protein encoded by the PRND gene, located near the PRNP gene.
  • Human Dpl shares structural similarities with cellular prion protein (PrPC) but exhibits distinct functions.
  • The PRND gene has varied expression patterns, with low levels in the adult central nervous system (CNS).

Purpose of the Study:

  • To investigate the functional differences between Doppel (Dpl) and PrPC.
  • To explore the role of the PRND gene and its polymorphic sequences in neurodegenerative disorders.
  • To clarify the function of Dpl protein in male gametogenesis and acrosome function.

Main Methods:

  • Comparative analysis of Dpl and PrPC structures.
  • Gene expression analysis of Prnd in mouse embryos and adult tissues.
  • Investigation of Dpl's inability to enhance pathological prion protein (PrPSc) propagation.
  • Examination of PRND polymorphic sequences and their correlation with neurodegenerative diseases.

Main Results:

  • Dpl protein is not capable of enhancing the propagation of the pathological prion protein (PrPSc).
  • Dpl protein regulates acrosome function and male gametogenesis.
  • Studies on PRND polymorphic sequences and their link to neurodegenerative diseases have yielded contradictory results.

Conclusions:

  • Despite structural similarities, Dpl and PrPC have different functions.
  • The role of the PRND gene in prion diseases like Creutzfeldt-Jakob disease (CJD) remains unclear.
  • Further research is needed to elucidate the exact function of Dpl and the implications of PRND gene variations.

More Related Videos

High-throughput Screening for Protein-based Inheritance in S. cerevisiae08:12

High-throughput Screening for Protein-based Inheritance in S. cerevisiae

Published on: August 8, 2017

RNAi Mediated Gene Knockdown and Transgenesis by Microinjection in the Necromenic Nematode Pristionchus pacificus06:57

RNAi Mediated Gene Knockdown and Transgenesis by Microinjection in the Necromenic Nematode Pristionchus pacificus

Published on: October 16, 2011

Related Concept Videos

Interpreting ¹H NMR Signal Splitting: The (n + 1) Rule01:10

Interpreting ¹H NMR Signal Splitting: The (n + 1) Rule

In the AX proton spin system, proton A can sense the two spin states of a coupled proton X, resulting in a doublet NMR signal with two peaks of equal (1:1) intensity. When proton A is coupled to two equivalent protons (AX2 spin system), the spin states of each X can be aligned with or against the external field, creating three possible scenarios. This results in a 1:2:1  triplet signal, where the central peak corresponds to the chemical shift of A and is twice as large or intense as the others.
Polytene Chromosomes02:04

Polytene Chromosomes

Polytene chromosomes are giant interphase chromosomes with several DNA strands placed side by side. They were discovered in the year 1881 by Balbiani in salivary glands, intestine, muscles, malpighian tubules, and hypoderm of larvae Chironomus plumosus. Hence, these are also called "Salivary gland chromosomes." These are found in insects of the order Diptera and Collembola; in certain organs of mammals; and synergids, antipodes of flowering plants. Polytene chromosomes are also regularly...
Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining, normally used to...
Pleiotropy01:33

Pleiotropy

Pleiotropy is the phenomenon in which a single gene impacts multiple, seemingly unrelated phenotypic traits. For example, defects in the SOX10 gene cause Waardenburg Syndrome Type 4, or WS4, which can cause defects in pigmentation, hearing impairments, and an absence of intestinal contractions necessary for elimination. This diversity of phenotypes results from the expression pattern of SOX10 in early embryonic and fetal development. SOX10 is found in neural crest cells that form melanocytes,...
Subviral Agents01:29

Subviral Agents

Subviral agents are infectious entities that resemble viruses but lack one or more viral components, such as a capsid or essential replication machinery. These agents include viroids, prions, and satellites, each possessing distinct structural and functional characteristics that influence their mode of infection and replication.Viroids are the simplest subviral agents, consisting of circular, single-stranded RNA molecules without a protein coat. They exclusively infect plants, relying entirely...
Insertion of Multi-pass Transmembrane Proteins in the RER01:29

Insertion of Multi-pass Transmembrane Proteins in the RER

The rough ER membrane synthesizes, assembles, and embeds transmembrane proteins in diverse topologies. These proteins function as transporters or channels and can remain in the ER membrane or are sent to the Golgi complex, lysosome, and cell membrane.
The multipass transmembrane proteins are the type IV integral membrane proteins with multiple topogenic sequences determining their spatial arrangement in the ER membrane. Nearly all multipass proteins lack a cleavable signal sequence and use...