What is an Ion Channelopathy?

Hello All,

Periodic Paralysis is a channelopathy and channelopathies are considered a class four metabolic disorder. Periodic Paralysis is a fourth class mineral metabolic disorder. Today's blog is about ion channelopathies.


What is an Ion Channelopathy?

Periodic Paralysis is a rare condition like no other. It is called an ion channelopathy, which is a dysfunction of an ion channel. Ion channelopathies were first recognized in 1971 and Periodic Paralysis was one of the first to be discovered. ^{1, 2}

 Ion channels are like a microscopic tunnel in the cells of muscles. The tunnels are called muscle fibers. Ions, which are molecules or atoms, flow in and out of the muscle cells through membranes or gates. Each of the gates is shaped exactly for the correct ion or molecule to enter. The ions are made up of what we call minerals, electrolytes or proteins. Some of the common ions are potassium, sodium, magnesium, chloride and calcium. They are electrically charged and each has its own size or shape, so to speak. If the gates or membranes are faulty in size or shape, an inefficient or improper flow through the membranes can and does cause muscle weakness and paralysis because they regulate contraction and relaxation of the muscle.

Through research, I have discovered that channelopathies are classified as a class four metabolic disorder. Disorders of metabolism are usually inherited and are involved in  chemical and physical processing, which use and make energy in the body. These processes include: breathing, circulation of blood, food and nutrient digestion, elimination of waste through bowel and bladder and temperature regulation.

Unfortunately, ion channelopathies are not usually categorized nor listed in medical writing or studies as metabolic disorders. This poses a problem for recognition, diagnosis and treatment by physicians and other medical professionals. I have found and heard Periodic Paralysis, which is a channelopathy, referred to as, a neuromuscular disease (affecting muscles and/or nerves also known as myoneural), a muscular dystrophy (wasting of muscle and eventual early death), and a disease of the nervous system (nothing to do with the nervous system), therefore, none of these is correct. Periodic Paralysis is a metabolic disorder, a condition which is based in the faulty cellular level of how energy is produced in our bodies. ³

To clarify, even further, Periodic Paralysis is a channelopathy which is a mineral metabolic disorder. Metabolism disorders involving minerals are conditions in which there is either not enough or an overabundance of minerals in an individual’s blood. Minerals have many functions in metabolism and the functions of the human body. They are important in bone and muscle building and growth. Organs, cells and tissues need minerals in order to function properly. So, a dysfunction of minerals in the body affects many processes and functions. Potassium, the main mineral involved in Periodic Paralysis, is involved in making proteins from the amino acids and plays a role in carbohydrate metabolism, so a dysfunction involving it, can affect more than just muscles. ^{1, 2}

 

1. Wikipedia. (February 2013) Ion Channel. Retrieved from:  

http://en.wikipedia.org/wiki/Ion_channel

2. Wikipedia. (December 2012). Channelopathy. Retrieved from: 

http://en.wikipedia.org/wiki/Channelopathy

3. WikiDoc. (August 2012). Metabolic disorder. Retrieved from: 

http://www.wikidoc.org/index.php/Metabolic_disorder

More information:Oxford Journals. (2002). Ion Channel Diseases. Retrieved from:

 http://hmg.oxfordjournals.org/content/11/20/2435.full

Added 1/25/2017

***An Introduction to Metabolic Disorder (Mineral metabolic disorder= Channelopathy)

http://www.encognitive.com/node/1181

A fourth class, the channelopathies (some of which cause periodic paralysis and/or malignant hyperthermia) could be considered to be metabolic disorders as well, though they are not always classified as such. These disorders affect the ion channels in the cell and organelle membranes, resulting in improper or inefficient transfer of ions through the membranes.


***Mineral Metabolism Disorders
http://careers.maimonidesmed.org/Main/AdamMultimediaEncyclopedia/Mineral-metabolism-disorders-1007271.aspx

Definition

Mineral metabolism disorders are abnormal levels of minerals -- either too much or too little -- in the blood.
Minerals are very important for the human body. They have various roles in metabolism and body functions. They are essential for the proper function of cells, tissues, and organs.
Some minerals, such as iron, make up part of many proteins and enzymes in the body. Others, such as potassium, help to produce proteins from amino acids and are involved in carbohydrate metabolism. Minerals also play a role in the building of muscle and bone and are important for normal body growth.
Metabolism refers to all the physical and chemical processes in the body that create and use energy, such as:

• Breathing
• Circulating blood
• Digesting food and nutrients
• Eliminating waste through urine and feces
• Regulating temperature

Causes

Disorders of mineral metabolism are sometimes passed from parents to their children through genes. Other medical conditions, such as starvation, diarrhea, or alcoholism, can cause mineral metabolism problems.

Disorders

Minerals that play a large role in the body include:

• Calcium
• Magnesium
• Phosphorus
• Potassium
• Selenium
• Sodium

Disorders in which mineral metabolism problems often occur include:

• Disorders of phosphorus metabolism:
• Hypophosphatemia
• Osteomalacia
• Rickets
• Rhabdomyolysis
• Hyperparathyroidism
• Hypoparathyroidism
• Disorders of potassium metabolism:
• Bartter syndrome
• Periodic paralysis with hypokalemia***
• Hypokalemic periodic paralysis***
• Hyperaldosteronism - primary and secondary
• Cushing’s disease
• Proximal renal tubular acidosis
• Distal renal tubular acidosis
• Fanconi’s syndrome
• Addison’s disease
• Kidney disease
• Disorders of iron metabolism:
• Hemochromatosis
• Cirrhosis
• Disorders of copper metabolism:
• Wilson’s disease
• Menkes syndrome
• Disorders of calcium metabolism:
• Hyperparathyroidism
• Nephrocalcinosis
• Pseudohypoparathyroidism
• Hypercalcemia
• Osteoporosis
• Movement - unpredictable or jerky
• Kidney stones
• Milk-alkali syndrome
• Paget’s disease
• Multiple endocrine neoplasia (MEN 1)
• Osteomalacia
• Rickets
• Disorders of sodium metabolism:
• Dilutional hyponatremia (SIADH)
• Hypernatremia
• Disorders of magnesium metabolism:
• Hypomagnesemia
• Hypermagnesemia
• Disorders of selenium metabolism
• Selenium deficiency
• Selenium excess
  
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Other Disorders Involving Metabolism Of Minerals











Public Health Service Publication No. 1693
https://books.google.com/books?id=icIaAAAAMAAJ&pg=PA153&lpg=PA153&dq=mineral+metabolism++disorders+periodic+paralysis&source=bl&ots=b-rjeyEFyj&sig=Dy_C8ZLRQGtzoqLoBRaxW1p9qQI&hl=en&sa=X&ved=0ahUKEwiY0Zy7j97RAhUQzWMKHbxFBDEQ6AEIUTAJ#v=onepage&q=mineral%20metabolism%20%20disorders%20periodic%20paralysis&f=false

Metabolic Disorder
http://www.wikidoc.org/index.php/Metabolic_disorder

Ion Channels list
http://www.nature.com/ncb/journal/v6/n11/fig_tab/ncb1104-1039_T1.html

PP IS A MINERAL METABOLIC DISORDER http://healthmedicinet.com/ency/article/007271.htm
Mineral metabolism disorders - Health Medicine Network healthmedicinet.com/ency/article/007271.htm

Until later...