Cockayne Syndrome (Syndrome, Cockayne)

A syndrome characterized by multiple system abnormalities including DWARFISM; PHOTOSENSITIVITY DISORDERS; PREMATURE AGING; and HEARING LOSS. It is caused by mutations of a number of autosomal recessive genes encoding proteins that involve transcriptional-coupled DNA REPAIR processes. Cockayne syndrome is classified by the severity and age of onset. Type I (classical; CSA) is early childhood onset in the second year of life; type II (congenital; CSB) is early onset at birth with severe symptoms; type III (xeroderma pigmentosum; XP) is late childhood onset with mild symptoms.
Also Known As:
Syndrome, Cockayne; Progeria Like Syndrome; Cockayne Syndrome Type 3; Cockayne Syndrome Type C; Cockayne Syndrome, Group A; Cockayne Syndrome, Group B; Cockayne Syndrome, Group C; Cockayne Syndrome, Type A; Cockayne Syndrome, Type B; Cockayne Syndrome, Type C; Cockayne Syndrome, Type I; Cockayne Syndrome, Type II; Cockayne Syndrome, Type III; Group A Cockayne Syndrome; Group B Cockayne Syndrome; Group C Cockayne Syndrome; Type A Cockayne Syndrome; Type B Cockayne Syndrome; Type C Cockayne Syndrome; Type I Cockayne Syndrome; Type II Cockayne Syndrome; Type III Cockayne Syndrome; Progeria-Like Syndromes; Syndrome, Progeria-Like; Progeria-Like Syndrome
Networked: 398 relevant articles (1 outcomes, 12 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Rare Diseases (Rare Disease)
2. Xeroderma Pigmentosum (Kaposi's Disease)
3. Neoplasms (Cancer)
4. Progeria (Hutchinson Gilford Syndrome)
5. Werner Syndrome (Werner's Syndrome)


1. Bohr, Vilhelm A: 22 articles (11/2014 - 02/2002)
2. Tanaka, Kiyoji: 12 articles (09/2015 - 07/2002)
3. Egly, Jean-Marc: 10 articles (02/2015 - 07/2004)
4. Sarasin, Alain: 9 articles (06/2015 - 09/2002)
5. Stevnsner, Tinna: 9 articles (01/2014 - 12/2002)
6. Berneburg, M: 8 articles (12/2012 - 01/2000)
7. Vermeulen, Wim: 7 articles (11/2014 - 09/2002)
8. Hoeijmakers, Jan H J: 7 articles (11/2014 - 07/2004)
9. Wilson, David M: 7 articles (11/2014 - 01/2007)
10. Kraemer, Kenneth H: 7 articles (07/2013 - 06/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Cockayne Syndrome:
1. Neurocutaneous melanosisIBA
2. Proteins (Proteins, Gene)IBA
3. TrichothiodystrophyIBA
4. DNA (Deoxyribonucleic Acid)IBA
5. Staphylococcal Protein A (A, Protein)IBA
6. Pyrimidine DimersIBA
7. UbiquitinIBA
8. Interferon-betaIBA
9. DNA-Formamidopyrimidine Glycosylase (Formamidopyrimidine DNA Glycosylase)IBA
10. Hydrogen Peroxide (Hydroperoxide)FDA LinkGeneric

Therapies and Procedures

1. Drug Therapy (Chemotherapy)
2. General Anesthesia
3. Deep Brain Stimulation
4. Cochlear Implantation
5. Fundoplication