UROPORPHYRINOGEN I SYNTHASE Meaning and
Definition
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Uroporphyrinogen I Synthase, also known as UROS, is an enzyme that plays a crucial role in the production of heme, the iron-containing molecule responsible for oxygen transportation in the body. It is an essential enzyme in the heme biosynthesis pathway, particularly in the formation of the first cyclic tetrapyrrole known as uroporphyrinogen I.
Uroporphyrinogen I Synthase catalyzes the conversion of hydroxymethylbilane, a linear tetrapyrrole intermediate, into uroporphyrinogen I. This is achieved through a series of complex chemical reactions involving multiple enzymatic steps. The enzyme is highly specific and requires various cofactors and metal ions for its activity.
Deficiencies or mutations in the UROS gene associated with Uroporphyrinogen I Synthase can lead to a rare genetic disorder known as congenital erythropoietic porphyria (CEP). CEP is characterized by the impaired production of heme, resulting in the accumulation of toxic intermediates called porphyrins. This condition manifests with various symptoms, including severe skin photosensitivity, abnormal red blood cell production, and occasional hepatosplenomegaly.
Understanding the structure and function of Uroporphyrinogen I Synthase is of significant interest in medical research to develop potential therapies for porphyrias and related disorders. Targeting this enzyme may help modulate heme synthesis and alleviate the symptoms associated with porphyrias. Furthermore, studying UROS may also offer insights into the broader field of tetrapyrrole metabolism and its implications for human health.
Common Misspellings for UROPORPHYRINOGEN I SYNTHASE
- yroporphyrinogen i synthase
- hroporphyrinogen i synthase
- jroporphyrinogen i synthase
- iroporphyrinogen i synthase
- 8roporphyrinogen i synthase
- 7roporphyrinogen i synthase
- ueoporphyrinogen i synthase
- udoporphyrinogen i synthase
- ufoporphyrinogen i synthase
- utoporphyrinogen i synthase
- u5oporphyrinogen i synthase
- u4oporphyrinogen i synthase
- uriporphyrinogen i synthase
- urkporphyrinogen i synthase
- urlporphyrinogen i synthase
- urpporphyrinogen i synthase
- ur0porphyrinogen i synthase
- ur9porphyrinogen i synthase
- urooorphyrinogen i synthase
- urolorphyrinogen i synthase
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