Stephen J. Schueler, M.D.

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Adrenoleukodystrophy Sex-Linked

Adrenoleukodystrophy is a X-linked genetic disease, meaning that the defective gene responsible for the disorder is on the X chromosome.

The letters X and Y represents the last pair of chromosomes. The last two chromosomes are called sex chromosomes, because they determine our gender. Females have two X-chromosomes in every cell, while males have one X-chromosome and one Y-chromosome. The X and Y-chromosomes contain portions that do not match. The genes on these portions are expressed without "competition" from a matching gene on another chromosome.

When gene mutations cause disease, they are referred to as genetic diseases. Gene mutations that are attached to one of the sex chromosomes (often the X chromosome) are referred to as sex-linked. When these mutations cause disease, they are often referred to as sex-linked disorders. If the mutation is on the X-chromosome, it is an X-linked disorder. Most X-linked disorders only affect males, because the unmatched genes on the X-chromosome are expressed without competition from the Y-chromosome.

Last Updated: Nov 4, 2008 References
Authors: Stephen J. Schueler, MD; John H. Beckett, MD; D. Scott Gettings, MD
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PubMed Adrenoleukodystrophy References
  1. Moser H, Dubey P, Fatemi A. Progress in X-linked adrenoleukodystrophy. Curr Opin Neurol. 2004 Jun;17(3):263-9. [1516705]
  2. Moser HW, Fatemi A, Zackowski K, Smith S, Golay X, Muenz L, Raymond G. Evaluation of therapy of X-linked adrenoleukodystrophy. Neurochem Res. 2004 May;29(5):1003-16. [15139299]
  3. Schiffmann R, van der Knaap MS. The latest on leukodystrophies. Curr Opin Neurol. 2004 Apr;17(2):187-92. [15021247]
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