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A Genetic Disease That Destroys The Myelin Sheath Of A Brain Neuron

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Affecting 1 in every 18,000 people, Adrenoleukodystrophy (ALD) is a genetic disease that destroys the myelin sheath surrounding a brain neuron. A brain neuron is an essential cell body that is responsible for muscle contractions and ultimately, our ability to move. Adrenoleukodystrophy is a devastating genetic mutation that affects X-chromosomes in both males and females. However, because males only have one X-chromosome, the outcome is catastrophic. As said, Adrenoleukodystrophy is a x-linked metabolic disorder that progressively breaks down the myelin sheath around a brain neuron. The myelin sheath is an insulating membrane that is responsible for allowing electrical impulses to transmit effectively through cell body’s. Without it, the brain can no longer relay messages to other systems in the body. The loss of myelin is accompanied by dysfunction of the adrenal gland and inability to move. The breakdown of the myelin sheath is caused from a mutation of the gene that makes the Adrenoleukodystrophy protein (ALDP). This ALD protein helps the body metabolize saturated very-long-chain fatty acids found in the serum and tissues of the central nervous system. The newly mutated gene no longer acts as a help aid to breaking down the long-chain fats. Therefore, the body starts accumulating an abnormal amount of fat in the nervous system, adrenal gland and testes that sets off an unusual response in the immune system; demyelination. Adrenoleukodystrophy is one of many genetic

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