What is glutathione?
What are the health benefits of glutathione?
Glutathione (gamma-glutamyl-cysteinyl-glycine) is the most abundant low-molecular-weight
thiol, and the "glutathione /glutathione disulfide" is the major redox couple in animal cells.

The synthesis of is catalyzed sequentially by gamma-glutamylcysteine synthetase and
glutathione synthetase. Glutathione synthesis is regulated primarily by gamma-glutamylcysteine
synthetase activity. Adequate protein nutrition is important for the maintenance of glutathione
homeostasis.

Thiol groups are kept in a reduced state. In effect, glutathione reduces any disulfide bond
formed within cytoplasmic proteins to cysteines by acting as an electron donor. In the process,
glutathione is converted to its oxidized form glutathione disulfide (GSSG). Glutathione is found
almost exclusively in its reduced form, since the enzyme that reverts it from its oxidized form,
glutathione reductase, is constitutively active and inducible upon oxidative stress. Some
people use the ratio of reduced glutathione to oxidized glutathione within cells as a measure of
cellular toxicity. [Wikipedia, March 2009]

What are the roles of glutathione in our health?

Glutathione plays important roles in antioxidant defense, nutrient metabolism, and regulation of
cellular events (including gene expression, DNA and protein synthesis, cell proliferation and
apoptosis, signal transduction, cytokine production and immune response, and protein
glutathionylation).

What is glutathione synthetase deficiency?

Glutathione synthetase deficiency is a disorder that prevents the production of an important
molecule called glutathione. Glutathione helps prevent damage to cells by neutralizing harmful
molecules generated during energy production. Glutathione also plays a role in processing
medications and cancer-causing compounds (carcinogens), and building DNA, proteins, and
other important cellular components.

Glutathione synthetase deficiency can be classified into three types: mild, moderate, and
severe. Mild glutathione synthetase deficiency usually results in the destruction of red blood
cells (hemolytic anemia). Rarely, affected people also excrete large amounts of a compound
called 5-oxoproline in their urine (5-oxoprolinuria). This compound builds up when glutathione
is not processed correctly in cells.

Individuals with moderate glutathione synthetase deficiency may experience symptoms
beginning shortly after birth including hemolytic anemia, 5-oxoprolinuria, and elevated acidity in
the blood and tissues (metabolic acidosis).

In addition to the features present in moderate glutathione synthetase deficiency, individuals
affected by the severe form of this disorder may experience neurological symptoms. These
problems may include seizures; a generalized slowing down of physical reactions, movements,
and speech (psychomotor retardation); intellectual disability; and a loss of coordination
(ataxia). Some people with severe glutathione synthetase deficiency also develop recurrent
bacterial infections.

What diseases are related to glutathione deficiency?

Glutathione deficiency contributes to oxidative stress, which plays a key role in aging and the
pathogenesis of many diseases (including kwashiorkor, seizure, Alzheimer's disease,
Parkinson's disease, liver disease, cystic fibrosis, sickle cell anemia, HIV, AIDS, cancer, heart
attack, stroke, and diabetes).

How common is glutathione synthetase deficiency?

Glutathione synthetase deficiency is very rare.

Sources [1] Glutathione synthetase deficiency ghr.nlm.nih.gov March 2009.
[2] Wu G, Fang YZ, Yang S, Lupton JR, Turner ND. Texas A&M University, Glutathione metabolism and its implications
for health. J Nutr. 2004 Mar;134(3):489-92.
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