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Adrenoleukodystrophy (Adrenoleukodystrophy, X-Linked)

An X-linked recessive disorder characterized by the accumulation of saturated very long chain fatty acids in the LYSOSOMES of ADRENAL CORTEX and the white matter of CENTRAL NERVOUS SYSTEM. This disease occurs almost exclusively in the males. Clinical features include the childhood onset of ATAXIA; NEUROBEHAVIORAL MANIFESTATIONS; HYPERPIGMENTATION; ADRENAL INSUFFICIENCY; SEIZURES; MUSCLE SPASTICITY; and DEMENTIA. The slowly progressive adult form is called adrenomyeloneuropathy. The defective gene ABCD1 is located at Xq28, and encodes the adrenoleukodystrophy protein (ATP-BINDING CASSETTE TRANSPORTERS).
Also Known As:
Adrenoleukodystrophy, X-Linked; Adrenomyeloneuropathy; X-Linked Adrenoleukodystrophy; ALD (Adrenoleukodystrophy); Addison Disease and Cerebral Sclerosis; Bronze Schilder Disease; Melanodermic Leukodystrophy; Siemerling-Creutzfeldt Disease; X-ALD; X-ALD (X-Linked Adrenoleukodystrophy); Leukodystrophies, Melanodermic; Leukodystrophy, Melanodermic; Schilder Addison Complex; Siemerling Creutzfeldt Disease; X ALD; X ALD (X Linked Adrenoleukodystrophy); X Linked Adrenoleukodystrophy; Schilder-Addison Complex
Networked: 1188 relevant articles (40 outcomes, 101 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Adrenoleukodystrophy (Adrenoleukodystrophy, X-Linked)
2. Metachromatic Leukodystrophy (Sulfatide Lipidosis)
3. Globoid Cell Leukodystrophy (Krabbe Disease)
4. Neurodegenerative Diseases (Neurodegenerative Disease)
5. Inborn Genetic Diseases (Disease, Hereditary)

Experts

1. Kemp, Stephan: 45 articles (01/2022 - 08/2002)
2. Pujol, Aurora: 34 articles (01/2022 - 12/2004)
3. Wanders, Ronald J A: 31 articles (12/2017 - 04/2002)
4. Raymond, Gerald V: 29 articles (01/2022 - 10/2002)
5. Berger, Johannes: 27 articles (01/2021 - 10/2002)
6. Moser, Ann B: 25 articles (10/2021 - 08/2002)
7. Orchard, Paul J: 24 articles (05/2022 - 08/2004)
8. Shimozawa, Nobuyuki: 24 articles (02/2022 - 10/2003)
9. Aubourg, Patrick: 23 articles (01/2021 - 03/2003)
10. Engelen, Marc: 21 articles (01/2022 - 01/2010)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Adrenoleukodystrophy:
1. Fatty Acids (Saturated Fatty Acids)IBA
2. Lorenzo's oilIBA
3. Proteins (Proteins, Gene)FDA Link
4. Biomarkers (Surrogate Marker)IBA
5. ATP-Binding Cassette Transporters (ABC Transporters)IBA
6. EnzymesIBA
7. AntioxidantsIBA
8. leriglitazoneIBA
9. lignoceric acid (tetracosanoic acid)IBA
10. Lysophosphatidylcholines (Lysophosphatidylcholine)IBA

Therapies and Procedures

1. Therapeutics
2. Hematopoietic Stem Cell Transplantation
3. Stem Cell Transplantation
4. Bone Marrow Transplantation (Transplantation, Bone Marrow)
5. Transplantation