UQCRB: Difference between revisions

Latest revision as of 20:39, 30 December 2024

Ubiquinol-cytochrome c reductase binding protein (UQCRB) is a protein that in humans is encoded by the UQCRB gene. This protein is a component of the mitochondrial electron transport chain, specifically part of Complex III (also known as cytochrome bc1 complex). It plays a crucial role in the process of oxidative phosphorylation, which is essential for ATP synthesis in eukaryotic cells.

Function[edit]

UQCRB is involved in the binding of ubiquinol and the transfer of electrons from ubiquinol to cytochrome c. This process is vital for the generation of an electrochemical gradient across the inner mitochondrial membrane, which drives the synthesis of ATP by ATP synthase.

Clinical significance[edit]

Mutations in the UQCRB gene have been associated with various mitochondrial disorders, which can lead to a range of clinical symptoms due to impaired energy production. These disorders often affect tissues with high energy demands, such as muscle and nervous tissue.

Research[edit]

Studies have shown that UQCRB may also play a role in angiogenesis, the process of new blood vessel formation, which is important in both normal physiological processes and in pathological conditions such as cancer.

References[edit]

External links[edit]

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-'''UQCRB''' (Ubiquinol-Cytochrome C Reductase Binding Protein) is a protein that in humans is encoded by the UQCRB gene. It is a component of the [[ubiquinol-cytochrome c reductase]] complex (complex III or cytochrome b-c1 complex), which is part of the [[mitochondrial respiratory chain]]. This protein plays an essential role in cellular respiration, a process that generates ATP, the cell's main source of energy.
+{{Infobox protein
+| name = Ubiquinol-cytochrome c reductase binding protein
+| image = <!-- Image removed -->
+| caption = <!-- Caption removed -->
+| symbol = UQCRB
+| altsymbols = QCR7
+| hgncid = 12588
+| omim = 191330
+| refseq = NM_006294
+| uniprot = P14927
+| chromosomal_location = 8q22
+}}
+'''Ubiquinol-cytochrome c reductase binding protein''' ('''UQCRB''') is a protein that in humans is encoded by the '''UQCRB''' gene. This protein is a component of the [[mitochondrial electron transport chain]], specifically part of [[Complex III]] (also known as [[cytochrome bc1 complex]]). It plays a crucial role in the process of [[oxidative phosphorylation]], which is essential for [[ATP synthesis]] in [[eukaryotic cells]].
 == Function ==
+UQCRB is involved in the binding of [[ubiquinol]] and the transfer of electrons from ubiquinol to [[cytochrome c]]. This process is vital for the generation of an [[electrochemical gradient]] across the [[inner mitochondrial membrane]], which drives the synthesis of ATP by [[ATP synthase]].
-The UQCRB protein is a subunit of the ubiquinol-cytochrome c reductase complex. This complex, which is located in the inner mitochondrial membrane, plays a crucial role in the mitochondrial respiratory chain by catalyzing the transfer of electrons from [[ubiquinol]] (coenzyme Q) to [[cytochrome c]]. This electron transfer is coupled with the translocation of protons across the inner mitochondrial membrane, contributing to the establishment of a proton gradient that drives ATP synthesis.
+== Clinical significance ==
+Mutations in the UQCRB gene have been associated with various [[mitochondrial disorders]], which can lead to a range of clinical symptoms due to impaired energy production. These disorders often affect tissues with high energy demands, such as [[muscle]] and [[nervous tissue]].
-== Clinical Significance ==
-Mutations in the UQCRB gene have been associated with [[mitochondrial complex III deficiency]], a rare genetic disorder characterized by a broad spectrum of clinical manifestations, including growth retardation, liver dysfunction, hypoglycemia, and lactic acidosis. Early-onset forms of the disease can lead to severe neurological symptoms, such as [[encephalopathy]], [[seizures]], and [[hypotonia]].
 == Research ==
+Studies have shown that UQCRB may also play a role in [[angiogenesis]], the process of new blood vessel formation, which is important in both normal physiological processes and in [[pathological conditions]] such as [[cancer]].
-Research on UQCRB has focused on understanding its role in the mitochondrial respiratory chain and its implications in disease. Studies have shown that UQCRB is essential for the assembly and stability of the ubiquinol-cytochrome c reductase complex. Furthermore, research has suggested that UQCRB could be a potential therapeutic target for diseases related to mitochondrial dysfunction.
+== See also ==
+* [[Mitochondrial diseases]]
-[[File:UQCRB protein structure.jpg|thumb|right|300px|The 3D structure of the UQCRB protein. Image from Wikimedia Commons.]]
+* [[Electron transport chain]]
-== See Also ==
-* [[Mitochondrial disease]]
 * [[Oxidative phosphorylation]]
-* [[Electron transport chain]]
 == References ==
+<references />
-<references />
+== External links ==
+* [https://www.ncbi.nlm.nih.gov/gene/7382 UQCRB gene - NCBI]
+* [https://www.uniprot.org/uniprot/P14927 UQCRB protein - UniProt]
+{{Mitochondrial proteins}}
+{{Electron transport chain}}
-[[Category:Proteins]]
+[[Category:Human proteins]]
-[[Category:Genes]]
+[[Category:Genes on chromosome 8]]
-[[Category:Mitochondrial diseases]]
+[[Category:Mitochondrial proteins]]
-[[Category:Biochemistry]]
+[[Category:Electron transport chain]]
-{{Protein-stub}}
-{{medicine-stub}}