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Adenosine diphosphate (ADP), also known as adenosine pyrophosphate (APP), is an important organic compound in metabolism and is essential to the flow of energy in living cells. ADP consists of three important structural components: a sugar backbone attached to adenine and two phosphate groups bonded to the 5 carbon atom of ribose .
Myogenic hyperuricemia, as a result of the purine nucleotide cycle running when ATP reservoirs in muscle cells are low (ADP > ATP), is a common pathophysiologic feature of glycogenoses such as GSD-III, GSD-V and GSD-VII, as they are metabolic myopathies which impair the ability of ATP (energy) production within muscle cells.
ADP/ATP translocase 1 is the major AAC in human cells and the archetypal protein of this family. It has a mass of approximately 30 kDa, consisting of 297 residues. [ 12 ] It forms six transmembrane α-helices that form a barrel that results in a deep cone-shaped depression accessible from the outside where the substrate binds.
The ATP-ADP translocase (also called adenine nucleotide translocase, ANT) is an antiporter and exchanges ADP and ATP across the inner membrane. The driving force is due to the ATP (−4) having a more negative charge than the ADP (−3), and thus it dissipates some of the electrical component of the proton electrochemical gradient.
Activated PARP can deplete the cell of ATP in an attempt to repair the damaged DNA. ATP depletion in a cell leads to lysis and cell death (necrosis). [16] [17] PARP also has the ability to induce programmed cell death, via the production of PAR, which stimulates mitochondria to release AIF. [18] This mechanism appears to be caspase-independent.
AMP does not have the high energy phosphoanhydride bond associated with ADP and ATP. AMP can be produced from ADP by the myokinase (adenylate kinase) reaction when the ATP reservoir in the cell is low: [5] [6] 2 ADP → ATP + AMP. Or AMP may be produced by the hydrolysis of one high energy phosphate bond of ADP: ADP + H 2 O → AMP + P i
The adenylate energy charge is an index used to measure the energy status of biological cells.. ATP or Mg-ATP is the principal molecule for storing and transferring energy in the cell : it is used for biosynthetic pathways, maintenance of transmembrane gradients, movement, cell division, etc...
ADP from the cytosol is transported back into the mitochondrion for ATP synthesis and the synthesised ATP, produced from oxidative phosphorylation, is exported out of the mitochondrion for use in the cytosol, providing the cells with its main energy currency. [7] TOM: Translocase of the outer membrane. Mitochondrial import receptor subunit TOM20.