What does ATP synthase gene code for?
ATP synthase is composed of two linked multi-subunit complexes: the soluble catalytic core, F1, and the membrane-spanning component, Fo, comprising the proton channel. This gene encodes the alpha subunit of the catalytic core.
Is ATP synthase a gene?
ATP synthase is a protein that catalyzes the formation of the energy storage molecule adenosine triphosphate (ATP) using adenosine diphosphate (ADP) and inorganic phosphate (Pi)….
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What gene makes ATP?
Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase or Complex V) produces ATP from ADP in the presence of a proton gradient across the membrane which is generated by electron transport complexes of the respiratory chain.
Where is ATP synthase located in the mitochondrion?
inner mitochondrial membrane
Human mitochondrial (mt) ATP synthase, or complex V consists of two functional domains: F1, situated in the mitochondrial matrix, and Fo, located in the inner mitochondrial membrane.
What does human ATP synthase do?
The ATP synthase in human mitochondria provides most cellular ATP under aerobic conditions. Energy derived from oxidative metabolism generates a proton-motive force (pmf) across the inner membrane of the organelle, and the ATP synthase harnesses the pmf to make ATP from ADP and phosphate (1, 2).
What happens if the ATP gene is mutated?
A mutation at nucleotide position 8993 of human mitochondrial DNA, located within the gene for ATP synthase subunit 6, is associated with the neurological muscle weakness, ataxia, and retinitis pigmentosa (NARP) syndrome.
What causes low ATP?
Complex changes in mitochondrial structure and function, including disorganization of mitochondrial structure, decline in the activity of enzymes involved in mitochondrial ATP synthesis, accumulation of mtDNA mutations, increased damage of mitochondrial proteins and lipids by reactive oxygen species are considered to …
How does ATP synthase work?
ATP synthase is a huge molecular complex (>500,000 daltons) embedded in the inner membrane of mitochondria. Its function is to convert the energy of protons (H+) moving down their concentration gradient into the synthesis of ATP. One ATP synthase complex can generate >100 molecules of ATP each second.
Why is ATP synthase important?
The function of ATP synthase is to produce ATP. ATP is necessary to power all cellular processes, so it is constantly being used by cells and constantly needs to be produced. Each ATP synthase can produce about 100 molecules of ATP every second.
What happens if ATP synthase is defective?
The insufficient ATP synthase phosphorylating capacity with respect to the respiratory chain results in an impaired energy provision and an increased ROS production due to an elevated mitochondrial membrane potential (1,16).
What is the synthesis of ATP?
ATP synthesis involves the transfer of electrons from the intermembrane space, through the inner membrane, back to the matrix. The combination of the two components provides sufficient energy for ATP to be made by the multienzyme Complex V of the mitochondrion, more generally known as ATP synthase.
What is the function of the ATP5F1A gene?
ATP synthase F1 subunit alpha, mitochondrial is an enzyme that in humans is encoded by the ATP5F1A gene. This gene encodes a subunit of mitochondrial ATP synthase. Mitochondrial ATP synthase catalyzes ATP synthesis, using an electrochemical gradient of protons across the inner membrane during oxidative phosphorylation.
Are there any antibodies that can detect ATP5A1?
Antibodies that detect ATP5A1 can be used in several scientific applications, including Western Blot, Immunocytochemistry, Immunohistochemistry (Paraffin), Immunohistochemistry and Immunoprecipitation. View more This gene encodes a subunit of mitochondrial ATP synthase.
How does mitochondrial ATP synthase catalyze ATP synthesis?
Mitochondrial ATP synthase catalyzes ATP synthesis, using an electrochemical gradient of protons across the inner membrane during oxidative phosphorylation. ATP synthase is composed of two linked multi-subunit complexes: the soluble catalytic core, F 1, and the membrane-spanning component, F o, comprising the proton channel.
Which is the most frequently affected subunit of ATP5F1A?
ATP5F1A, a complex V protein, was the most frequently affected subunit Using systems biology techniques to study gene coexpression network, ATP5A1 was identified and found highly expressed in normal kidney than clear cell renal cell carcinoma (ccRCC) tissues of each grade.