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Hypokalemic Periodic Paralysis II. BME 301 Spring 2008 Dr. Entcheva Group 6. Hypokalemic Periodic Paralysis II: Symptoms. Weakness Paralysis . Temporal duration: Up to 24 hours. Hypokalemic Periodic Paralysis II: Factors. Mutation of SCN4A gene Situational:

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hypokalemic periodic paralysis ii

Hypokalemic Periodic Paralysis II

BME 301 Spring 2008

Dr. Entcheva

Group 6

hypokalemic periodic paralysis ii symptoms
Hypokalemic Periodic Paralysis II: Symptoms

Weakness Paralysis

Temporal duration: Up to 24 hours

hypokalemic periodic paralysis ii factors
Hypokalemic Periodic Paralysis II: Factors

Mutation of

SCN4A gene

Situational:

  • Excess of carbohydrates, salt, alcohol
  • Immediate rest after strenuous activity
  • Sudden temperature change
hypokalemic periodic paralysis ii action potential
Hypokalemic Periodic Paralysis II: Action Potential

*

*Jurkat-Rott, Karin, et al. Voltage-sensor sodium channel mutations cause hypokalemic periodic paralysis type 2 by enhanced inactivation and reduced current. PNAS. 2000 Aug; 97(17):9549-54.

hypokalemic periodic paralysis ii treatment
Hypokalemic Periodic Paralysis II: Treatment

Preventative

During Attack

Hospitalization

Potassium

  • Diet low in Na and carbohydrates
  • Avoidance of attack-inducing situations
      • Acetazolamide (Diamox)

Understanding through Modeling…

hypokalemic periodic paralysis ii modeling
Hypokalemic Periodic Paralysis II: Modeling

Model of diseased

cell’s electrical activity using

Matlab

  • Creation of Graphical User Interface (GUI)
  • Changes to code supplied by Dr. Entcheva
changes to tau h
Changes to Tau h

Tau_h in HypoPP is about 65% of the normal value

Tauh =.65

No changes to Tau_m or Tau_j values  Tauj = Taum = 1

hypokalemic periodic paralysis ii sodium gate
Hypokalemic Periodic Paralysis II: Sodium Gate

*Taken from Dr. Entcheva’s BME 301 Spring 2008 in-class lecture.

hypopp2 affects skeletal muscle not cardiac muscle
HypoPP2 affects Skeletal MuscleNOT CARDIAC MUSCLE!!

*Jurkat-Rott, Karin, et al. Voltage-sensor sodium channel mutations cause hypokalemic periodic paralysis type 2 by enhanced inactivation and reduced current. PNAS. 2000 Aug; 97(17):9549-54.

hypokalemic periodic paralysis ii diseased cardiac ventricular cell simulation
Hypokalemic Periodic Paralysis II: Diseased CardiacVentricular CellSimulation

HypoPP2

Normal Cell

references
References

1. Mosenkis, Ari. Hypokalemic periodic paralysis. U.S. National Library of Medicine. 01 March 2006. <http://www.nlm.nih.gov/medlineplus/ency/article/000312.htm>.

2. Sternberg, Damien, Thierry Maisonobe, and Karin Jurkat-Rott. "Hypokalaemic Periodic Paralysis Type 2 Caused by Mutations At Codon 672 in the Muscle Sodium Channel Gene SCN4A." Brain: a Journal of Neurology 124 (2001): 1091-1099. PubMed. 21 Mar. 2008.

3. Jurkat-Rott, Karin, et al. “Voltage-sensor sodium channel mutations cause

hypokalemic periodic paralysis type 2 by enhanced inactivation and reduced current.” PNAS. 2000 Aug; 97(17):9549-54.

4. Bulman DE, Scoggan KA, van Oene MD, Nicolle MW, Hahn AF, Tollar LL, et al. A novel sodium channel mutation in a family with hypokalemic periodic paralysis. Neurology 1999; 53: 1932–6.

5. Sansone, V et al. Treatment for periodic paralysis. Cochrane Database Syst Rev. 2008 Jan 23;(1)

6. Hypokalemic Periodic Paralysis: A Model for a Clinical and Research Approach to a Rare Disorder.  Neurotherapeutics, Volume 4, Issue 2, Pages 225-232 B. Fontaine, E. Fournier, D. Sternberg, S. Vicart, N. Tabti