It takes the average reader 2 hours and 19 minutes to read X-linked Adrenoleukodystrophy by Doriane Trompier
Assuming a reading speed of 250 words per minute. Learn more
X-linked adrenoleukodystrophy (X-ALD) is the most common leukodystrophy and the most frequent peroxisomal disorder, with an estimated incidence of 1:17,000. This complex neurodegenerative disorder is characterized by a huge clinical variability both in the age of onset and in symptoms. The two main forms are the childhood cerebral ALD (ccALD) characterized by inflammatory demyelination of the central nervous system, and the adult form called adrenomyeloneuropathy (AMN), characterized by a non-inflammatory slowly progressive demyelination affecting spinal cord and peripheral nerves. Adrenal insufficiency is usually associated with the nervous symptoms, X-ALD being the main cause of Addison's disease. The main biochemical defect is the accumulation of very-long-chain fatty acids (VLCFA, fatty acids with a chain length of more than 22 carbon atoms), particularly in the cholesterol ester fraction of the adrenal gland and brain white matter. X-ALD is associated with mutations in the ABCD1 gene, which encodes for a peroxisomal ATPBinding Cassette (ABC) transporter predicted to allow VLCFA-CoA to enter into the peroxisome for their β-oxidation. In spite of animal models, the physiopathogenesis of the disease remains poorly understood. However, several therapeutic strategies have been investigated. Among them, allogeneic bone marrow transplantation has proven effective and, more recently, the first gene therapy trial ended with a resounding success.
X-linked Adrenoleukodystrophy by Doriane Trompier is 136 pages long, and a total of 34,816 words.
This makes it 46% the length of the average book. It also has 43% more words than the average book.
The average oral reading speed is 183 words per minute. This means it takes 3 hours and 10 minutes to read X-linked Adrenoleukodystrophy aloud.
X-linked Adrenoleukodystrophy is suitable for students ages 10 and up.
Note that there may be other factors that effect this rating besides length that are not factored in on this page. This may include things like complex language or sensitive topics not suitable for students of certain ages.
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