SPR Human
Description
Sepiapterin Reductase is expressed with a 20 amino acid His tag at N-Terminus and purified by proprietary chromatographic techniques.
Product Specs
Product Science Overview
Sepiapterin reductase (SPR) is a crucial enzyme in the biosynthesis of tetrahydrobiopterin (BH4), a cofactor essential for the activity of several enzymes, including nitric oxide synthases and aromatic amino acid hydroxylases. The human recombinant form of this enzyme has been extensively studied due to its significant role in various biological processes and its association with multiple diseases.
SPR is a homodimer composed of two subunits. It catalyzes the final step in the biosynthesis of BH4, converting sepiapterin to 7,8-dihydrobiopterin, which is subsequently reduced to BH4. This process is vital for the proper functioning of enzymes involved in neurotransmitter synthesis, such as phenylalanine hydroxylase, tyrosine hydroxylase, and tryptophan hydroxylase .
SPR exhibits a wide distribution in different tissues, including the brain, liver, and cardiovascular system. In the brain, SPR is localized in the pyramidal neurons of the cerebral cortex, striatal neurons, and neurons of the hypothalamic and brainstem monoaminergic regions . The expression of SPR is also notably high in certain cancers, such as liver and colorectal cancer .
The dysfunction or deficiency of SPR is associated with several diseases. For instance, SPR deficiency can lead to a rare metabolic disorder characterized by a deficiency in BH4, resulting in hyperphenylalaninemia and neurotransmitter deficiencies. This condition can manifest as developmental delays, movement disorders, and other neurological symptoms .
Moreover, SPR has been implicated in chronic pain, cardiovascular diseases, and cancer. The enzyme’s role in these conditions is thought to be linked to its involvement in the regulation of nitric oxide production and neurotransmitter synthesis .
Given its critical role in various biological processes, SPR is a potential target for therapeutic interventions. Several compounds have been identified that can modulate SPR activity, offering potential treatment options for diseases associated with SPR dysfunction . For example, the administration of L-DOPA or 5-hydroxytryptophan in combination with carbidopa has been shown to improve symptoms in patients with SPR deficiency .
