Wolff-Parkinson-White syndrome affects 1 to 3 in 1,000 people worldwide. Only a small fraction of these cases appear to run in families. Many cases were found in UK and in Boston after world war 2 . India ,Pakistan and europe is the has reported very less cases as per the data get from cardic books ,
Wolff-Parkinson-White syndrome is a common cause of an arrhythmia known as paroxysmal supraventricular tachycardia. Wolff-Parkinson-White syndrome is the most frequent cause of this abnormal heart rhythm in the Chinese population, where it is responsible for more than 70 percent of cases.
Prkag2-gene--The official name of this gene is “protein kinase, AMP-activated, gamma 2 non-catalytic subunit.
Where is the PRKAG2 gene located?
Cytogenetic Location: 7q36.1 Molecular Location on chromosome 7: base pairs 151,253,199 to 151,574,315http://spermup.blogspot.com/
The PRKAG2 gene is located on the long (q) arm of CHROMOSOME 7 at position 36.1.
More precisely, the PRKAG2 gene is located from base pair 151,253,199 to base pair 151,574,315 on chromosome 7.
Mutations in the PRKAG2 gene cause Wolff-Parkinson-White syndrome.
Mutations( A mutation is a permanent change in the DNA sequence of a gene. Mutations in a gene's DNA sequence can alter the amino acid sequence of the protein encoded by the gene.
How does this happen? Like words in a sentence, the DNA sequence of each gene determines the amino acid sequence for the protein it encodes. The DNA sequence is interpreted in groups of three nucleotide bases, called codons. Each codon specifies a single amino acid in a protein.)
A small percentage of all cases of Wolff-Parkinson-White syndrome are caused by mutations in the PRKAG2 gene. Some people with these mutations also have features of hypertrophic cardiomyopathy, a form of heart disease that enlarges and weakens the heart (cardiac) muscle. The PRKAG2 gene provides instructions for making a protein that is part of an enzyme called AMP-activated protein kinase (AMPK). This enzyme helps sense and respond to energy demands within cells. It is likely involved in the development of the heart before birth, although its role in this process is unknown.
Researchers are uncertain how PRKAG2 mutations lead to the development of Wolff-Parkinson-White syndrome and related heart abnormalities. Research suggests that these mutations alter the activity of AMP-activated protein kinase in the heart, although it is unclear whether the genetic changes overactivate the enzyme or reduce its activity. Studies indicate that changes in AMP-activated protein kinase activity allow a complex sugar called glycogen to build up abnormally within cardiac muscle cells. Other studies have found that altered AMP-activated protein kinase activity is related to changes in the regulation of certain ion channels in the heart. These channels, which transport positively charged atoms (ions) into and out of cardiac muscle cells, play critical roles in maintaining the heart's normal rhythm.
In most cases, the cause of Wolff-Parkinson-White syndrome is unknown
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