Extracellular Vesicles as Potential Biomarkers and Therapeutic Target in Gaucher Disease

Description

This is an observational study intended to generate preliminary data to understand how lysosomal dysfunction can affect the biogenesis of extracellular vesicles, its content and function. The primary objective of the proposed project is to decipher how extracellular vesicle (EV) biogenesis and its role in intercellular communication can be impaired as a consequence of defects in lysosomal function. Collectively these defects in EV biogenesis and function can contribute to the neuroinflammation observed in lysosomal storage diseases. Since EVs can cross the blood-brain barrier, their characterization may be valuable in identifying novel biomarkers. In the presence of a GBA1 mutation, the decrease in GCase activity will lower overall lysosome function and increase the secretion of EVs. Further, there will be differences in EV size, its cargo including lipids, RNA and proteins and their aggregates. In comparison to healthy controls, EVs isolated from patients with Gaucher disease (GD) and GBA1 carriers is hypothesized to show significant differences in terms of its characteristics and content, which can contribute to our understanding of the link between lysosomes and neurological disease.

Conditions

Gaucher Disease

Study Overview

Study Details

Study overview

This is an observational study intended to generate preliminary data to understand how lysosomal dysfunction can affect the biogenesis of extracellular vesicles, its content and function. The primary objective of the proposed project is to decipher how extracellular vesicle (EV) biogenesis and its role in intercellular communication can be impaired as a consequence of defects in lysosomal function. Collectively these defects in EV biogenesis and function can contribute to the neuroinflammation observed in lysosomal storage diseases. Since EVs can cross the blood-brain barrier, their characterization may be valuable in identifying novel biomarkers. In the presence of a GBA1 mutation, the decrease in GCase activity will lower overall lysosome function and increase the secretion of EVs. Further, there will be differences in EV size, its cargo including lipids, RNA and proteins and their aggregates. In comparison to healthy controls, EVs isolated from patients with Gaucher disease (GD) and GBA1 carriers is hypothesized to show significant differences in terms of its characteristics and content, which can contribute to our understanding of the link between lysosomes and neurological disease.

Extracellular Vesicles as Potential Biomarkers and Therapeutic Target in Gaucher Disease

Extracellular Vesicles as Potential Biomarkers and Therapeutic Target in Gaucher Disease

Condition
Gaucher Disease
Intervention / Treatment

-

Contacts and Locations

Minneapolis

University of Minnesota, Minneapolis, Minnesota, United States, 55414

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

For general information about clinical research, read Learn About Studies.

Eligibility Criteria

  • * Age between 18-80yrs
  • * Restricted to participants who are untreated, obligate carriers and healthy controls.
  • * Participants with GD should have confirmed GD diagnosis, mutation confirmed for carriers and healthy controls confirmed to have no GBA1 mutation by gene sequencing.
  • * Exclude participants who have any hematological malignancy or other uncontrolled comorbid conditions.
  • * Exclude participants who are currently on therapy for their GD
  • * Exclude participants who have any hematological malignancy or other uncontrolled comorbid conditions.

Ages Eligible for Study

18 Years to 80 Years

Sexes Eligible for Study

ALL

Accepts Healthy Volunteers

No

Collaborators and Investigators

University of Minnesota,

Reena Kartha, PhD, MS, PRINCIPAL_INVESTIGATOR, University of Minnesota

Subbaya Subramanian, PhD, MS, PRINCIPAL_INVESTIGATOR, University of Minnesota

Study Record Dates

2025-05