Related Experiment Video
Updated: Aug 14, 2026
10:32
Assessing Functional Performance in the Mdx Mouse Model
Published on: March 28, 2014
Utrophin: a structural and functional comparison to dystrophin
D J Blake1, J M Tinsley, K E Davies
1Department of Biochemistry, University of Oxford, UK.
Brain Pathology (Zurich, Switzerland)
|January 1, 1996
Summary
Utrophin, similar to dystrophin, may replace it in Duchenne muscular dystrophy (DMD). This review explores utrophin
Area of Science:
- Biochemistry
- Molecular Biology
- Neuroscience
Background:
- Utrophin is an autosomal homologue of dystrophin, the protein affected in Duchenne muscular dystrophy (DMD).
- While structurally similar, utrophin and dystrophin exhibit reciprocal expression patterns in skeletal muscle.
- Utrophin is primarily found at the neuromuscular junction (NMJ), co-localizing with acetylcholine receptors (AChR).
Purpose of the Study:
- To compare the structure and function of utrophin and dystrophin.
- To discuss the potential of utrophin as a therapeutic agent for DMD.
Main Methods:
- Literature review comparing utrophin and dystrophin.
- Analysis of expression patterns and functional roles.
Main Results:
- Utrophin shares significant sequence similarity and protein-binding properties with dystrophin.
- Utrophin expression increases and localizes to the sarcolemma in certain myopathies, including DMD.
- Utrophin's role at the NMJ involves potential stabilization of the synaptic cytoskeleton.
Conclusions:
- Utrophin's structural and functional similarities suggest it could compensate for dystrophin deficiency.
- Utrophin represents a promising therapeutic candidate for Duchenne muscular dystrophy.
Related Concept Videos
01:56Structural Protein Function
Structural proteins are a category of proteins responsible for functions ranging from cell shape and movement to providing support to major structures such as bones, cartilage, hair, and muscles. This group includes proteins such as collagen, actin, myosin, and keratin.
Collagen, the most abundant protein in mammals, is found throughout the body. In connective tissue, such as skin, ligaments, and tendons, it provides tensile strength and elasticity. In bones and teeth, it mineralizes to form...
Collagen, the most abundant protein in mammals, is found throughout the body. In connective tissue, such as skin, ligaments, and tendons, it provides tensile strength and elasticity. In bones and teeth, it mineralizes to form...
01:26Introduction to Actin
Actin is a highly conserved cytoskeletal protein found abundantly in eukaryotic cells. It constitutes 10% weight of the total cellular protein in muscle cells, while in non-muscle cells, it is lower and makes up around 1–5 percent of the total cell protein. Actin found in the unicellular amoebae and complex multicellular animals is around 80% similar, demonstrating their conservation over a billion years of evolution. Actin coding genes are conserved within species and across different species.
01:15Overview of Myosin Structure and Function
Myosins are a family of molecular motor proteins, first identified in the skeletal muscles, where they are responsible for muscle contraction. Along with their role in muscle contraction, these proteins also play a role in the intracellular transport of molecules and vesicles. There are twenty-four classes of myosins based on their domain sequence and organization. Of the twenty-four, six classes (Myosin I, Myosin II, Myosin V, Myosin VI, Myosin VII, and Myosin X) have been well characterized.
01:18ATP Synthase: Structure
ATP synthase or ATPase is among the most conserved proteins found in bacteria, mammals, and plants. This enzyme can catalyze a forward reaction in response to the electrochemical gradient, producing ATP from ADP and inorganic phosphate. ATP synthase can also work in a reverse direction by hydrolyzing ATP and generating an electrochemical gradient. Different forms of ATP synthases have evolved special features to meet the specific demands of the cell. Based on their specific feature, ATP...
01:56Structural Protein Function
Structural proteins are a category of proteins responsible for functions ranging from cell shape and movement to providing support to major structures such as bones, cartilage, hair, and muscles. This group includes proteins such as collagen, actin, myosin, and keratin.
Collagen, the most abundant protein in mammals, is found throughout the body. In connective tissue, such as skin, ligaments, and tendons, it provides tensile strength and elasticity. In bones and teeth, it mineralizes to form...
Collagen, the most abundant protein in mammals, is found throughout the body. In connective tissue, such as skin, ligaments, and tendons, it provides tensile strength and elasticity. In bones and teeth, it mineralizes to form...
01:08The Sarcomere
A sarcomere is a microscopic segment repeating in a myofibril. The sarcomere fundamentally consists of two main myofilaments: thick filaments called myosin and thin filaments called actin. These filaments interact by sliding past each other in response to stimulus. In addition to myosin and actin, several other proteins, such as tropomyosin, troponin, titin, nebulin, myomesin, α-actinin, and dystrophin, play crucial roles in regulating, structuring, and functioning of the sarcomere.
Each myosin...
Each myosin...