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Genetic basis for WPW syndrome

Genetic basis for WPW syndrome

Genetic basis for WPW syndrome: Not all cases of WPW syndrome may have a genetic basis. One of the genes to be implicated first in WPW syndrome was  PRKAG2 gene which encodes for the gamma-2 regulatory subunit of AMP-activated protein kinase. The enzyme functions by inactivating key enzymes involved in regulating de novo biosynthesis of fatty acid and cholesterol. Mutations in the gene causes distortion of the atrioventricular annulus by glycogen-filled myocytes and causes ventricular pre-excitation. The gene location has been given as 7q36.1 in the OMIM (Online Mendelian Inheritance in Man®) database.

BMP2 was another gene which has been linked with WPW syndrome by S R Lalani, J V Thakuria, G F Cox, X Wang, W Bi, M S Bray, C Shaw, S W Cheung, A C Chinault, B A Boggs, Z Ou, E K Brundage, J R Lupski, J Gentile, S Waisbren, A Pursley, L Ma, M Khajavi, G Zapata, R Friedman, J J Kim, J A Towbin, P Stankiewicz, S Schnittger, I Hansmann, T Ai, S Sood, X H Wehrens, J F Martin and J W Belmont, L Potocki [1]. Bone morphogenetic protein is involved in the development of the atrioventricular canal. Microdeletion of 20p12.3 produces pre-excitation (Wolff-Parkinson-White syndrome) along with variable neurocognitive deficits.

Reference

  1. S R Lalani, J V Thakuria, G F Cox, X Wang, W Bi, M S Bray, C Shaw, S W Cheung, A C Chinault, B A Boggs, Z Ou, E K Brundage, J R Lupski, J Gentile, S Waisbren, A Pursley, L Ma, M Khajavi, G Zapata, R Friedman, J J Kim, J A Towbin, P Stankiewicz, S Schnittger, I Hansmann, T Ai, S Sood, X H Wehrens, J F Martin, J W Belmont, L Potocki. 20p12.3 microdeletion predisposes to Wolff-Parkinson-White syndrome with variable neurocognitive deficits. J Med Genet 2009;46:168 –75.