Definition
A mercaptoethylamine compound that is endogenously derived from the COENZYME A degradative pathway. The fact that cysteamine is readily transported into LYSOSOMES where it reacts with CYSTINE to form cysteine-cysteamine disulfide and CYSTEINE has led to its use in CYSTINE DEPLETING AGENTS for the treatment of CYSTINOSIS. Cysteamine is a product of the constitutive degradation of coenzyme A, a process that occurs in all tissues, although some tissues such as brain and heart may have exceptionally high coenzyme A turnover rates. Cysteamine has only one known function, and that is as a precursor for the formation of hypotaurine, which is subsequently oxidized to taurine. The rate of cysteamine production as a result of coenzyme A breakdown is not well understood but it is clear that cysteamine levels are not as dramatically affected by dietary habits as are cysteine levels. Cysteamine is generated from hypotaurine by cysteamine dioxygenase (EC:1.13.11.19), an enzyme that was recently identified in mammals (PMID: 17581819). Cysteamine is the simplest stable aminothiol found in the body. It is used in the treatment of disorders of cystine excretion. Cysteamine cleaves the disulfide bond with cysteine to produce molecules that can escape the metabolic defect in cystinosis and cystinuria. Cyst(e)amine may also serve as an endogenous regulator of immune system activity as well as a potential therapeutic agent for the treatment of Huntington disease. Cysteamine is also used as a radiation-protective agent that oxidizes in air to form cystamine. It can be given intravenously or orally to treat radiation sickness. -- Wikipedia [HMDB]
Top Gene Interactions
Related Pathways
Cysteamine (Procysbi) Interacts with Diseases
Disease | Inference Score | References/Inference Genes |
Reperfusion Injury | 17.19 | |
Pulmonary Emphysema | 16.68 |
|
Neoplasm Invasiveness | 15.39 | |
Esophageal Neoplasms | 15.03 |
References/Inference Genes
|
Diabetes Mellitus, Experimental | 12.9 | |
Rheumatoid arthritis | 12.41 | |
Obesity | 12.17 | |
Chronic obstructive pulmonary disease | 12.08 |
|
Ulcerative colitis | 11.49 |
References/Inference Genes
|
Prostatic Neoplasms | 11.33 | |
Adrenoleukodystrophy | 11.16 |
|
Colitis | 10.98 |
References/Inference Genes
|
Marfan Syndrome | 10.61 |
|
Ischemia | 10.5 |
References/Inference Genes
|
Hepatocellular carcinoma | 10.17 | |
Brain Ischemia | 9.79 |
References/Inference Genes
|
Carcinoma, Non-Small-Cell Lung | 9.74 |
|
Alcoholic liver cirrhosis | 9.71 |
|
Alzheimer's Disease | 9.64 |
|
Pancreatic carcinoma | 9.57 |
|