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Related Concept Videos

Gene Therapy00:59

Gene Therapy

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Gene therapy is a technique where a gene is inserted into a person’s cells to prevent or treat a serious disease. The added gene may be a healthy version of the gene that is mutated in the patient, or it could be a different gene that inactivates or compensates for the patient’s disease-causing gene. For example, in patients with severe combined immunodeficiency (SCID) due to a mutation in the gene for the enzyme adenosine deaminase, a functioning version of the gene can be...
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Pleiotropy01:33

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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,...
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Unrenewable Cells00:50

Unrenewable Cells

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In humans, the photoreceptor cells of the eye and sensory hair cells of the ear lack stem cells. These cells are thus unrenewable and cannot be replaced when they are damaged or destroyed.
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Overview
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In-vitro Mutagenesis01:16

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To learn more about the function of a gene, researchers can observe what happens when the gene is inactivated or “knocked out,” by creating genetically engineered knockout animals. Knockout mice have been particularly useful as models for human diseases such as cancer, Parkinson’s disease, and diabetes.
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Genetic Screens02:46

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Genetic screens are tools used to identify genes and mutations responsible for phenotypes of interest. Genetic screens help identify individuals or a group of people at risk of developing  genetic diseases and help them with early intervention, targeted therapy, and reproductive options.
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Related Experiment Video

Updated: Jun 6, 2025

Posterior Semicircular Canal Approach for Inner Ear Gene Delivery in Neonatal Mouse
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Gene therapy for hereditary hearing loss.

Zeming Fu1, Liping Zhao2, Yingyuan Guo1

  • 1Department of Otolaryngology- Head and Neck Surgery, The Second Hospital of Jilin University, 4026 Yatai Street, Changchun 130022, China.

Hearing Research
|December 1, 2024
PubMed
Summary

Gene therapy offers hope for hereditary hearing loss (HHL) by correcting genetic mutations. Adeno-associated virus (AAV) vectors show promise in delivering gene therapies, restoring hearing in some cases.

Keywords:
Adeno-associated virusCRISPR/CasGene therapyHereditary hearing loss

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Area of Science:

  • Genetics
  • Otolaryngology
  • Molecular Biology

Background:

  • Hereditary hearing loss (HHL) affects millions, with genetic factors implicated in about 50% of profound cases.
  • Over 150 genes are linked to hearing loss, highlighting the complexity of genetic deafness.
  • Gene therapy presents a potential solution for treating diseases caused by specific genetic mutations.

Purpose of the Study:

  • To review recent advances in gene therapy for hereditary hearing loss (HHL) based on animal models.
  • To explore various gene therapy strategies, including gene-editing tools, delivery vectors, and administration routes for HHL.
  • To discuss the strengths, limitations, and future challenges of clinical gene therapy applications for HHL.

Main Methods:

  • Review of current literature on gene therapy for HHL, focusing on animal model studies.
  • Analysis of CRISPR/Cas gene-editing technologies and their application in HHL research.
  • Evaluation of viral vectors, particularly adeno-associated virus (AAV), for inner ear gene delivery.

Main Results:

  • CRISPR/Cas gene editing has accelerated the development of gene therapies for HHL.
  • Adeno-associated virus (AAV) vectors have proven safe and effective for delivering genes to inner ear cells in animal models.
  • AAV-mediated gene therapy has shown potential to restore hearing in children with hereditary deafness.

Conclusions:

  • Gene therapy, particularly using AAV vectors, holds significant promise for treating hereditary hearing loss.
  • Further research is needed to identify specific HHL types and optimize gene therapy approaches for clinical use.
  • Addressing the limitations and challenges of current gene therapy methods is crucial for successful clinical translation in HHL treatment.