Effect of pharmacological chaperones on brain tyrosine hydroxylase and tryptophan hydroxylase 2

Phenylalanine Hydroxylase, Tyrosine Hydroxylase, and Tryptophan Hydroxylase

Zebrafish tyrosine hydroxylase 2 gene encodes tryptophan hydroxylase.

The primary pathological hallmark of Parkinson disease (PD) is the profound loss of dopaminergic neurons in the substantia nigra pars compacta. To facilitate the understanding of the underling mechanism of PD, several zebrafish PD models have been generated to recapitulate the characteristics of dopaminergic (DA) neuron loss. In zebrafish studies, tyrosine hydroxylase 1 (th1) has been frequentl...

Phenylalanine Hydroxylase Misfolding and Pharmacological Chaperones†

Phenylketonuria (PKU) is a loss-of-function inborn error of metabolism. As many other inherited diseases the main pathologic mechanism in PKU is an enhanced tendency of the mutant phenylalanine hydroxylase (PAH) to misfold and undergo ubiquitin-dependent degradation. Recent alternative approaches with therapeutic potential for PKU aim at correcting the PAH misfolding, and in this respect pharma...

The effect of Aerobic Training on Serotonin and Tryptophan Hydroxylase of Prefrontal Cortex in type 2 Diabetic Rats

Background & Aims: Type 2 diabetes (T2D) is a self-management disease and depression is a common problem related to it. One of the causes of depression is serotonin (5-HT) depleted. The enzyme tryptophan hydroxylase (TPH) is known as limiting enzyme in the production of 5-HT in the brain. Aerobic exercise also has proven benefits in treating and reducing the incidence of chronic diseases such a...

Tryptophan hydroxylase 1 knockout and tryptophan hydroxylase 2 polymorphism: effects on hypoxic pulmonary hypertension in mice.

Serotonin [5-hydroxytryptamine (5-HT)] biosynthesis depends on two rate-limiting tryptophan hydroxylases (Tph): Tph1, which is expressed in peripheral organs, and Tph2, which is expressed in neurons. Because 5-HT is involved in pulmonary hypertension (PH), we investigated whether genetic variations in Tph1 and/or Tph2 affected PH development in mice. To examine the functional impact of peripher...