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General Information Adrenoleukodystrophy ALD is a term used to refer to a genetic disorder that disrupts the adrenal glands due to the removal of white matter from the nerve fibers in an individuals brain which causes neuro-degeneration or deterioration The main cause of ALD would be a mutation in the gene that codes for a protein that is involved in a metabolic process The onset of this genetic disorder may occur during two stages of a male affected by adrenoleukodystrophy During childhood between ages four and eight the ALD sufferer will develop symptoms that usually include attention deficit disorder seizures and impairment of the senses and then those symptoms are followed by complete motor disability within two years The disorder may be referred to as adrenomyeloneuropathy if the symptoms such as progressive paraparesis sphincter disturbances and varying degrees of distal sensory loss are manifested during adulthood so the AMN affected individual only suffers from mild symptoms compared to children who have ALD There are a few gross anatomy issues that ALD causes including peroxisomal ATP-binding cassette ABC protein malfunction central nervous system deterioration and non-functioning adrenal glands Normally the human cell takes saturated very long chain fatty acids VLCFA into an organelle known as the peroxisome with an ABC transporter protein In the peroxisome the VLCFAs are broken down into an acetyl-CoA molecule fragment and another carbon chain attached to coenzyme a CoA However a person with ALD is unable to break down the VLCFAs into two smaller fragments due to the fact that an adrenoleukodystrophy protein ALDP exists instead of another functional ABC protein As VLCFAs build up throughout the body the central nervous system begins to deteriorate since these fatty acids will damage the white electrical insulation wrapping myelin sheath that is wrapped around the axons Consequently the individual experiences a vegetable state and she will also lose control of the adrenal glands that are responsible for releasing several hormones
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