Related Experiment Video
Updated: Jul 24, 2026

An In Vitro Model for the Study of Cellular Pathophysiology in Globoid Cell Leukodystrophy
Published on: October 21, 2014
Adrenoleukodystrophy: unusual clinical and radiographic manifestation
G A Williams1, G S Pearl, M A Pollack
1Department of Pathology, Orlando Regional Healthcare System, Fla., USA.
Abstract:
Adrenoleukodystrophy is an X-linked recessive peroxisomal disorder, characterized by progressive neurologic deterioration due to cerebral white matter demyelination and adrenal insufficiency. Onset is usually in childhood between ages 5 and 10, and its course is fatal within approximately 5 years. Initial symptoms are behavioral, gait, and auditory disturbances and may be a diagnostic dilemma. Abnormally raised plasma very long chain fatty acids (VLCFA) are diagnostic; computed tomography and magnetic resonance imaging findings show symmetrical occipital white matter lesions which progress in a rostralcaudal direction.
Related Concept Videos
Lysosomal Hydrolases
Smooth Endoplasmic Reticulum
The ER provides optimal conditions for synthesizing steroid hormones and lipids, such as phospholipids and triglycerides. Traditionally, lipid metabolism was considered to be a smooth ER function. However, there is no direct evidence to prove that rough ER is completely excluded from lipid...
Aneurysm II: Clinical Manifestations and Diagnostic Studies
Acute Kidney Injury III: Clinical Manifestations
Chronic Kidney Disease II: Clinical Manifestations
Inflammatory Bowel Disease IV: Clinical Manifestations

