UQCRH

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Protein UQCRH PDB 1bcc

UQCRH

UQCRH, also known as Ubiquinol-Cytochrome c Reductase Hinge Protein, is a protein that plays a crucial role in the electron transport chain within mitochondria. It is a component of the ubiquinol-cytochrome c reductase complex, also known as Complex III, which is a key enzyme in the process of oxidative phosphorylation.

Structure

UQCRH is a transmembrane protein that spans the inner mitochondrial membrane. It contains multiple domains that are involved in binding to other components of the ubiquinol-cytochrome c reductase complex and facilitating the transfer of electrons during the electron transport chain.

Function

The primary function of UQCRH is to serve as a hinge protein that connects the cytochrome b and cytochrome c1 subunits within Complex III. This connection is essential for the efficient transfer of electrons from ubiquinol to cytochrome c, ultimately generating a proton gradient that drives ATP synthesis.

Clinical Significance

Mutations in the gene encoding UQCRH have been associated with mitochondrial disorders and respiratory chain deficiencies. These conditions can lead to a variety of symptoms, including muscle weakness, exercise intolerance, and metabolic abnormalities.