Related Experiment Video

Updated: Feb 14, 2026

Preparation and In Vitro Characterization of Magnetized miR-modified Endothelial Cells
09:58

Preparation and In Vitro Characterization of Magnetized miR-modified Endothelial Cells

Published on: May 2, 2017

8.1K

A mouse model of miR-96, miR-182 and miR-183 misexpression implicates miRNAs in cochlear cell fate and homeostasis

Michael D Weston1, Shikha Tarang2, Marsha L Pierce3

  • 1Department of Oral Biology, School of Dentistry, Creighton University, 780729 California Plaza, Omaha, NE 68178-0729, USA. michaelweston@creighton.edu.

Scientific Reports
|February 25, 2018
PubMed

Insights

Germline mutations in microRNA 96 (miR-96) cause hereditary hearing loss. Overexpressing the miR-183 cluster in mice led to progressive hearing loss and hair cell degeneration, revealing key regulatory networks.

Area of Science:

  • Genetics
  • Neuroscience
  • Molecular Biology

Background:

  • Germline mutations in miR-96 are linked to hereditary hearing loss in humans and mice.
  • MicroRNAs (miRNAs) are crucial regulators of gene expression, impacting cellular differentiation and homeostasis.
  • The miR-183 cluster (miR-96, miR-182, miR-183) is co-expressed and plays a role in neurosensory development.

Purpose of the Study:

  • To develop a mouse model overexpressing the miR-183 cluster in the inner ear.
  • To identify target genes and biological processes regulated by the miR-183 cluster.
  • To understand the role of this miRNA cluster in cochlear homeostasis and neurosensory cell function.

Main Methods:

  • Generation of transgenic mice (Tg1MDW) overexpressing the miR-183 cluster in the inner ear.
  • Histological analysis of cochlear hair cells (inner hair cells [IHCs] and outer hair cells [OHCs]).
  • mRNA microarray analysis to identify downregulated genes in the cochlea.
  • Auditory Brainstem Response (ABR) and Distortion Product Otoacoustic Emission (DPOAE) tests to assess hearing function.

Main Results:

  • Transgenic mice (Tg1MDW/1MDW) exhibited a modest increase in IHCs initially but developed progressive neurosensory hearing loss.
  • Histological assessments revealed massive losses of both IHCs and OHCs in homozygotes.
  • Microarray data showed statistically significant enrichment of predicted miRNA target sites among downregulated genes, indicating miRNA-mediated regulation.
  • Hearing loss correlated with hair cell degeneration over time.

Conclusions:

  • The Tg1MDW mouse model effectively recapitulates miRNA-misexpression-induced hearing loss.
  • The miR-183 cluster plays a critical role in maintaining cochlear homeostasis and neurosensory cell integrity.
  • This model serves as a valuable tool for elucidating miRNA-regulated gene networks involved in auditory development and function.

More Related Videos

Tear-Derived Exosomal miR-15a as New Diagnostic Tool for Diabetic Retinopathy07:45

Tear-Derived Exosomal miR-15a as New Diagnostic Tool for Diabetic Retinopathy

Published on: December 30, 2025

446
Characterization of Functionally Associated miRNAs in Glioblastoma and their Engineering into Artificial Clusters for Gene Therapy09:40

Characterization of Functionally Associated miRNAs in Glioblastoma and their Engineering into Artificial Clusters for Gene Therapy

Published on: October 4, 2019

6.0K

Related Concept Videos

What is Homeostasis?01:16

What is Homeostasis?

Maintaining homeostasis requires that the body continuously maintain its internal conditions. Each physiological condition has a particular set point, from body temperature to blood pressure to levels of certain nutrients. A set point is the physiological value around which the normal range fluctuates. A normal range is a restricted set of values that is optimally healthful and stable. For example, the set point for normal human body temperature is approximately 37°C (98.6°F).
57.4K
pH Homeostasis01:31

pH Homeostasis

Acid-base homeostasis is essential for maintaining normal physiological activities in humans. The pH of various body fluids is strictly regulated because it is critical for the optimal activity of enzymes involved in metabolic reactions. Enzymes are basically proteins, so, any significant change in pH can affect their structure and activity. In humans, pH is regulated using three primary mechanisms— chemical buffer systems, respiratory regulation, and renal regulation.
Respiratory...
20.1K
Fates of Pyruvate01:20

Fates of Pyruvate

Pyruvate is the end product of glycolysis, where glucose is oxidized to pyruvate, simultaneously reducing NAD+ to NADH. Two molecules of ATP are also produced by substrate-level phosphorylation.
In aerobic organisms, pyruvate is metabolized via the citric acid cycle to produce reduced coenzymes NADH and FADH2. These coenzymes are then oxidized in the electron transport chain to produce ATP and, in the process, regenerate the NAD+ and FAD. As seen in some cell types and organisms, fermentation...
11.2K
Skeleton and Calcium Homeostasis01:21

Skeleton and Calcium Homeostasis

Calcium is not only the most abundant mineral in bone but also the most abundant mineral in the human body. Calcium ions are needed for bone mineralization, tooth health, heart rate regulation and strength of contraction, blood coagulation, the contraction of smooth and skeletal muscle cells, and the regulation of nerve impulse conduction. The average calcium level in the blood is about 10 mg/dL. When the body cannot maintain this level, a person will experience hypo or hypercalcemia.
6.1K
Personal Choice and Fate Attributions01:19

Personal Choice and Fate Attributions

Some individuals interpret life events as a consequence of their personal choices and actions, while others believe that outcomes are dictated by fate or destiny. This divergence in perspective has been examined in psychological and cross-cultural studies, particularly in relation to religious faith and cultural beliefs about causality.Fate and Personal ResponsibilityPeople who emphasize personal responsibility view events as direct consequences of their decisions. For instance, breaking a leg...
182
Glucose Homeostasis: Regulation of Blood Glucose01:02

Glucose Homeostasis: Regulation of Blood Glucose

Carbohydrates consumed through foods are converted into glucose, a crucial energy source for the body. In the prandial state, high blood glucose levels stimulate the secretion of insulin from the pancreas. Insulin inhibits hepatic glucose production and stimulates glucose uptake and metabolism by muscle and adipose tissue. The excess glucose is converted into glycogen and stored in the liver and muscles.
During fasting, when blood glucose levels are low, the pancreas secretes glucagon. it...
4.4K