HOMEPRODUCTSCOMPANYCONTACTFAQResearchDictionaryPharmaSign Up FREE or Login

Hepatolenticular Degeneration (Wilson's Disease)

A rare autosomal recessive disease characterized by the deposition of copper in the BRAIN; LIVER; CORNEA; and other organs. It is caused by defects in the ATP7B gene encoding copper-transporting ATPase 2 (EC 3.6.3.4), also known as the Wilson disease protein. The overload of copper inevitably leads to progressive liver and neurological dysfunction such as LIVER CIRRHOSIS; TREMOR; ATAXIA and intellectual deterioration. Hepatic dysfunction may precede neurologic dysfunction by several years.
Also Known As:
Wilson's Disease; Wilson Disease; Copper Storage Disease; Hepatic Form of Wilson Disease; Hepato-Neurologic Wilson Disease; Hepatocerebral Degeneration; Hepatolenticular Degeneration Syndrome; Kinnier-Wilson Disease; Progressive Lenticular Degeneration; Westphal-Strumpell Syndrome; Wilson Disease, Hepatic Form; Cerebral Pseudoscleroses; Copper Storage Diseases; Degeneration Syndrome, Hepatolenticular; Degeneration Syndromes, Hepatolenticular; Degeneration, Hepatocerebral; Degeneration, Hepatolenticular; Degeneration, Neurohepatic; Degeneration, Progressive Lenticular; Degenerations, Hepatocerebral; Degenerations, Neurohepatic; Disease, Copper Storage; Diseases, Copper Storage; Diseases, Hepato-Neurologic Wilson; Diseases, Kinnier-Wilson; Hepato Neurologic Wilson Disease; Hepato-Neurologic Wilson Diseases; Hepatocerebral Degenerations; Hepatolenticular Degeneration Syndromes; Kinnier Wilson Disease; Kinnier-Wilson Diseases; Lenticular Degeneration, Progressive; Neurohepatic Degenerations; Pseudoscleroses, Cerebral; Pseudosclerosis, Cerebral; Storage Disease, Copper; Storage Diseases, Copper; Syndrome, Hepatolenticular Degeneration; Syndromes, Hepatolenticular Degeneration; Westphal Strumpell Syndrome; Westphal-Strumpell Syndromes; Wilson Disease, Hepato-Neurologic; Wilson Diseases, Hepato-Neurologic; Wilsons Disease; Cerebral Pseudosclerosis; Neurohepatic Degeneration; Pseudosclerosis
Networked: 3939 relevant articles (146 outcomes, 331 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Liver Diseases (Liver Disease)
2. Inborn Genetic Diseases (Disease, Hereditary)
3. Cystinuria
4. Rheumatoid Arthritis
5. Hepatolenticular Degeneration (Wilson's Disease)

Experts

1. Członkowska, Anna: 55 articles (09/2022 - 02/2006)
2. Litwin, Tomasz: 54 articles (09/2022 - 04/2008)
3. Lutsenko, Svetlana: 51 articles (01/2022 - 01/2002)
4. Stremmel, Wolfgang: 43 articles (12/2021 - 09/2004)
5. Weiss, Karl Heinz: 42 articles (01/2022 - 04/2006)
6. Ferenci, Peter: 39 articles (01/2022 - 02/2002)
7. Schilsky, Michael L: 33 articles (12/2021 - 02/2002)
8. Brewer, George J: 29 articles (12/2015 - 03/2002)
9. Woimant, France: 25 articles (11/2022 - 03/2006)
10. Merle, Uta: 25 articles (12/2021 - 04/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hepatolenticular Degeneration:
1. CopperIBA
2. ZincIBA
3. Penicillamine (Cuprimine)FDA Link
4. Chelating AgentsIBA
5. tetrathiomolybdate (ammonium tetrathiomolybdate)IBA
6. Trientine (Trien)FDA Link
7. Zinc AcetateFDA Link
8. Ceruloplasmin (Ferroxidase)IBA
9. SaltsIBA
10. Proteins (Proteins, Gene)FDA Link

Therapies and Procedures

1. Therapeutics
2. Liver Transplantation
3. Transplantation
4. Chelation Therapy (Therapy, Chelation)
5. Plasma Exchange