Spinal Muscular Atrophy
Spinal Muscular Atrophy (SMA) is a motor neuron disease. The motor neurons affect the voluntary muscles used for crawling, walking, head and neck control and swallowing. It is a relatively common "rare disorder": approximately 1 in 6000 babies born are affected, and about 1 in 40 people are genetic carriers.
SMA affects muscles throughout the body, although proximal muscles (those closest to the trunk of one's body i.e. shoulders, hips and back) are often most severely affected. Weakness in the legs is generally greater than in arms. Sometimes feeding and swallowing can be affected. Involvement of respiratory muscles (muscles involved in breathing and coughing) can lead to an increased tendency for pneumonia and other lung problems. Sensation and the ability to feel are not affected. Intellectual activity is normal and often observed that patients with SMA are unusually bright and sociable.
Spinal Muscular Atrophy (SMA) is grouped under neurodegenerative diseases. Neurodegenerative disorders are a heterogeneous group of diseases of nervous system, including brain, spinal cord, and peripheral nerves, that have many different aetiologies. Many are hereditary, some are secondary to toxic or metabolic processes, and others result from infections. Neuropathologically, these are characterised by abnormalities of relatively specific regions of brain and specific populations of neurons. The degenerating neuron clusters in different diseases determine clinical phenotype of that particular illness.
Free Radicals : Free radicals are highly reactive molecules or chemical species capable of independent existence. Generation of highly Reactive Oxygen Species (ROS) is an integral feature of normal cellular function like mitochondrial respiratory chain, phagocytosis, arachidonic acid metabolism, ovulation and fertilisation. Their production however, multiplies several folds during pathological conditions.
Oxygen, because of its bi-radical nature, readily accepts unpaired electrons to give rise to a series of partially reduced species collectively known as ROS (Reactive Oxygen Species). Damage due to free radicals caused by ROS leads to several damaging effects as they can attack lipids, protein/ enzymes, carbohydrates and DNA in cells and tissues. They induce undesirable oxidation, causing membrane damage, protein modification, DNA damage and cell death induced by DNA fragmentation and lipid peroxidation.
Antioxidant systems
Endogenous antioxidants : Biological systems have evolved with endogenous defense mechanisms to help protect against free radical induced cell damage. Glutathione peroxidase, catalase and superoxide dismutases are antioxidant enzymes, which metabolize toxic oxidative intermediates. They require micronutrients as cofactors like selenium, iron, copper, zinc and manganese for optimum catalytic activity and effective antioxidant defense mechanisms. Glutathione, ascorbic acid, alpha-tocopherol, betacarotene, bilirubin, selenium, NADPH, butylhydroxyanisole (BHA), mannitol, benzoate, histidine peptide, the iron-bonding transferrin, dihydrolipoic acid, reduced CoQ10, melatonin, uric acid and plasma protein thiol, etc., as a whole play a homoeostatic or protective role against ROS produced during normal cellular metabolism and after active oxidation insult.
Exogenous antioxidants : The most widely studied dietary antioxidants are vitamin C, vitamin E, and beta-carotene. Vitamin C is the most important water-soluble antioxidant in extracellular fluids, as it is capable of neutralising ROS in the aqueous phase before lipid peroxidation is initiated. Vitamin E is a major lipid-soluble antioxidant, and is the most effective chain-breaking antioxidant within the cell membrane where it protects membrane fatty acids from lipid peroxidation. Beta-carotene and other carotenoids also provide antioxidant protection to lipid rich tissues.
A number of other dietary antioxidants exist beyond traditional vitamins collectively known as phytonutrients or phytochemicals which are being increasingly appreciated for their antioxidant activity. One example is flavonoids which are a group of polyphenolic compounds.
Oxidative Stress in the Nervous System : The nervous system including the brain, spinal cord and peripheral nerves is rich in both unsaturated fatty acids and iron. The high lipid content of nervous tissue, coupled with its high aerobic metabolic activity, makes it particularly susceptible to oxidative damage. The high level of iron may be essential, particularly during brain development, but its presence also means that injury to brain cells may release iron ions, which lead to oxidative stress via the iron-catalysed formation of ROS. In addition, those brain regions that are rich in catecholamines are exceptionally vulnerable to free radical generation. The catecholamine adrenaline, noradrenaline and dopamine can spontaneously break down (auto-oxidise) to free radicals or can be metabolized to radicals by the endogenous enzymes like MAO (monoamine oxidases).
A number of in vitro studies show that antioxidants both endogenous and dietary protect nervous tissue from damage by oxidative stress. Vitamin E prevents cell death (apoptosis) in rat neurons subjected to hypoxia followed by oxygen reperfusion. In the same study, it was shown that vitamin E prevented neuronal damage from reactive nitrogen species. Both vitamin E and beta carotene were found to protect rat neurons against oxidative stress from exposure to ethanol. In an experimental model of diabetes-caused neurovascular dysfunction, beta-carotene protects cells most effectively, followed by vitamin E and vitamin C.
There is substantial evidence that oxidative stress is a causative or at least ancillary factor in the pathogenesis of major neurodegenerative diseases, including Parkinson's disease, Alzheimer's disease, and amyotrophic lateral sclerosis (ALS, “Lou Gehrig's disease”) and in cases of stroke, trauma, and seizures. Decreased levels of antioxidant enzyme activity are reported in patients with Parkinson's disease. Evidence of increase in lipid peroxidation and oxidation of DNA and proteins has indeed been seen in the substantia nigra of patients affected with Parkinson's disease. Similar increases in markers of oxidative stress have also been seen in Alzheimer's disease, Huntington's disease and in both familial ALS (FALS) and sporadic ALS (SALS) patients.
The role of free-radical-mediated oxidative injury in acute insults to the nervous system including stroke or trauma, and in chronic neurodegenerative disorders, is being just recognised. As we know, oxygen is an essential molecule for survival of majority of living organisms. Oxidative stress is the harmful condition that occurs when there is excess of free radicals and/or a decrease in antioxidant levels. There is evidence to suggest that increase in energy metabolism by aerobic pathways enhances intracellular concentration of free oxygen radicals, which in turn enhance rate of the autocatalytic process of lipid peroxidation, inducing damage to brain structures, especially when physiological defences becomes insufficient or depleted. Antioxidants combat oxidative stress by working to neutralise excess free radicals and stopping them from starting the chain reactions that contribute to various diseases and premature aging. Evidence to date for oxidative stress in PD, TD, SCZ, AD and other neurodegenerative diseases is strongly persuasive. Clinical studies show that a number of events associated with Alzheimer's stimulate production of free radicals and depletion of antioxidant levels. Patients with Parkinson's also have reduced glutathione levels and free radical damage is found in the form of increased lipid peroxidation and oxidation of DNA bases.
Noni has the most powerful antioxidant properties which help a lot to protect our vital organs including brain from deadly attack of free radicals. Noni contains all the vitamins, rich in beta-carotenes, abundant source of trace minerals, flavonoids, besides more than 150 other beneficial micronutrients.
Antioxidant property of NONI can fight free radicals in three ways :
1. It prevents a free radical from forming.
2. It interrupts an oxidizing chain reaction to lessen effects of free radicals.
3. Antioxidants like Noni reduces the free radical's impact.
Noni as an antioxidant, neutralizes effects of free radicals, allowing the body to restore itself to proper balance leading to health and well-being. Noni contains many different synergistic antioxidant nutrients, including basic vitamins A,C & E. Drinking Noni every day combats damaging effects of free radicals.
The natural integrity of Noni is only part of the reason for its effectiveness. There are two additional reasons contributing to NONI's effectiveness for a broad range of conditions.
1. Unique Combinations of Substances
Noni has an impressive combination of ingredients. It has a rich complement of vitamins and minerals; including A, B vitamins (including the rare B-12), C, E, iron, calcium, sodium, potassium, zinc and several trace minerals. It also has 17 of 20 amino acids. Terpenes, fungicides, adaptogens, glycosides, polysaccharides and other unique ingredients that even in small amounts having significant physiological effects are found in Noni. It works like a food and acts like a medicine or herb without the side effects.
2. Synergy of its Substances
The many known, and even some of the unknown substances, come together in a way that supports needs of many of our internal systems, concurrently. As the word synergy implies, these substances work far better in combination than they do separately. Many of the ingredients in Noni are found in varying amounts in other foods or herbs. There seems to be no known food or herb with either the rich list of substances or the high amounts of key substances all put together in one super food. Noni aids the body's natural healing abilities.
Noni plays a vital role in preventing and also in some extent therapeutic role to many neurodegenerative diseases including SMA.
Recommended Dosage
Divine Noni Concentrate
5ml morning and 5ml evening for 3 days. Then
10ml morning and 10ml evening for next 3 days. Then
15ml morning and 15ml evening for next 15 days. Then
20ml morning and 20ml evening for next 8 months.
