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Dopamine Beta Hydroxylase Activity 1

Dopamine beta hydroxylase (DBH) is an enzyme crucial to the balance of dopamine and norepinephrine in the body.

Price: $475 (Testing + Consult) Discontinued 

No- But this test does require a blood draw at a local lab. 

Web results are posted within 7-14 business days. Our office will notify you when test results have been reported.

Yes. The kit comes with easy to follow instructions


Yes. Dr Hagmeyer will review the test result with you. Each test comes with a  post-test review/explanation.

One we have placed the order for the test we are unable to issue a refund.


Mental health disorders such as depression, anxiety, bipolar disorder, and schizophrenia have been documented to be associated with dysfunction in the DBH enzyme.  

Other conditions include postural orthostatic hypotension (POTS), exercise intolerance, fainting, autism spectrum disorder, ADHD, seizures, neurologic conditions, and alcoholism.



Dopamine beta hydroxylase (DBH)

Dopamine beta hydroxylase (DBH) is an enzyme crucial to the balance of dopamine and norepinephrine in the body.  It is required for the conversion of dopamine into norepinephrine.  

File:Dopamine beta-hydroxylase reaction.svg - Wikimedia Commons


DBH is like a lock associated with tyrosine and tyrosine derivatives fit like keys in the lock.  Dopamine, a derivative of tyrosine, fits on the DBH enzyme just as a key fits into a lock.  

DBH then transforms it into norepinephrine when it is functioning adequately, leaving us with proper levels of both dopamine and norepinephrine in the system.  

When DBH is functioning properly, we experience balanced moods and adequate energy levels.  When DBH dysfunction occurs, a wide range of abnormalities can develop.

During times of DBH dysfunction, no matter the cause, there will be a decreased conversion of dopamine to norepinephrine.  This causes a subsequent rise in dopamine levels and decrease in norepinephrine.  

Symptoms associated with elevated dopamine include anxiety, depression, fatigue, low blood pressure, sluggish deep tendon reflexes, ptosis, hypotonic muscles, hyperflexible reflexes, weakened facial muscles, and increased stress. Conditions associated with DBH dysfunction are wide ranging.  

Mental health disorders such as depression, anxiety, bipolar disorder, and schizophrenia have been documented to be associated with dysfunction in the DBH enzyme.  

Other conditions include postural orthostatic hypotension (POTS), exercise intolerance, fainting, autism spectrum disorder, ADHD, seizures, neurologic conditions, and alcoholism.

What happens when the autonomic nervous system malfunctions?

Dysautonomia is the term used to describe a dysfunction of the autonomic nervous system. Since this system regulates a large portion of the body’s functions, a variety of symptoms can be felt when it’s not working properly. Physical symptoms include dizziness, fatigue, fainting, chronic pain, headaches, abnormal sweating, shakiness, breathlessness, nausea, vision issues and more. There are many types of dysautonomia, but the most common are postural orthostatic tachycardia syndrome (POTS), orthostatic hypotension and neurocardiogenic syncope.

  • POTS: Patients with POTS have tachycardia (or a fast heart rate) when standing, due to inadequate blood flow in their bodies. A tilt table test is usually used to diagnosis the syndrome. During the test, the patient lays flat on a table as their heart rate, oxygen levels, and blood pressure is monitored. The table is then tilted slowly up to 90 degrees. Normally your heart rate should increase by around 10 – 20 beats per minute as your body adjusts to standing up. For adults, if your heart rate increases by more than 30 beats than you’re diagnosed with POTS. For children and adolescents, the heart rate would increase by more than 40 beats per minute.
  • Orthostatic Hypotension: If your blood pressure drops against gravity that is known as orthostatic hypotension. If your systolic, or the top number in your blood pressure, drops more than 20 mmHg or if the diastolic, the bottom number, drops more than 10 mmHg then you meet the criteria to be diagnosed with orthostatic hypotension.
  • Neurocardiogenic Syncope: Neurocardiogenic syncope (also known as vasovagal syncope) occurs when your heart rate and blood pressure drop and cause you to faint. This could be mild to severe in nature and may cause further issues, such as concussions which are common among people with neurocardiogenic syncope. A tilt table test is also used in the diagnosis of neurocardiogenic syncope.

The Missing link in Pots, Dysautonomia, Autism, ADHD THE MISSING and many other Neurological problems.


Dopamine beta-hydroxylase (DBH) is a key enzyme in the conversion of dopamine to norepinephrine in the central nervous system and is the enzyme needed for the conversion of dopamine to norepinephrine in the sympathetic nervous system of the autonomic nervous system.


Dopamine beta-hydroxylase can be readily measured in serum and correlates highly with the amount of the same enzyme in the cerebral spinal fluid.

The DBH gene has been mapped to chromosome 9 at the q34 position. Seventy-one polymorphisms of the DBH gene have been identified in just 80 subjects: 68 single nucleotide polymorphisms (SNPs), two insertion/deletions, and one repeat.

Because of the large number of SNPs, there are a very large number of possible combinations of variants for DBH (4,970 combinations). 

Abnormalities of DBH result in severe postural hypotension (POTS) and are also associated with the following symptoms and disorders:

  • Exercise Intolerance
  • Fainting Spells
  • Huntington’s Chorea
  • Hyperflexible Joints
  • Hypertension
  • Hypoglycemia
  • Impaired Ejaculation
  • Low Blood Pressure
  • Tourette’s Syndrome
  • Parkinson’s Disease
  • Psychosis
  • PTSD
  • Schizophrenia
  • Seizures
  • Spontaneous Abortion
  • Low Muscle Tone
  • Alcoholism
  • Autism Spectrum Disorders
  • Alzheimer’s Disease
  • Bed Wetting (Enuresis)
  • Bipolar Depression
  • Cardiac Heart Failure
  • Depression


Women with DBH deficiency are much more likely to have children with autism. In animals, DBH deficiency is associated with severe immune deficiency, impaired response to immunization, and lack of maternal care. It might be expected that similar abnormalities occur in humans.

Because of the very large number of possible combinations of variants for DBH and the fact that the amount of enzyme activity associated with each variant is unknown or reports on such SNPs are contradictory, assessment of DBH enzyme in serum or plasma is an excellent substitute for DNA testing.

Abnormalities in the metabolism of dopamine in autism consistent with DBH deficiency have been reported in the research literature for more than 40 years (Lelord 1978).

DBH deficiency greatly increases the concentration of dopamine and its metabolite homovanillic acid in the cerebrospinal fluid, blood, and urine because of the accumulation of dopamine when the DBH enzyme is less active.

Lelord, Callaway, Muh, Sauvage, and Arlot (1978) and Garreau et al. (1980) reported that urinary levels of the dopamine metabolite, homovanillic acid (HVA) were higher in autistic than in normal children, and that the clinical improvement of these young autistic patients under vitamin B6 was associated with a decrease of their urinary HVA excretion (Lelord, Muh, Barthelemy, Martineau, Garreau, & Callaway, 1981).

Abnormalities also included elevated concentrations of HVA in cerebrospinal fluid (Cohen, Caparulo, Shaywitz, & Bower, 1977, Gillberg).

In addition, the degree of elevation of HVA in urine was correlated with the increased severity of autistic symptoms (Garreau 1980).

The mean serum DBH was 30% lower in mothers of children with autism compared to mean values of those without a child with autism, indicating a likely genetic involvement of DBH as a cause of autism.

In the same study, it was found that mothers of sons with autism had a higher incidence of a 19 base pair deletion polymorphism (DBH-) in the DBH gene. The epidemiological term “attributable risk” for this gene was 42%, interpreted as meaning that the risk of having a son with autism was 42% higher for the mothers with this genetic variant than without it. DBH genotypes also differed significantly among mothers and controls, with 37% of mothers with two affected sons having two DBH− alleles, compared to 19% of controls.

The DBH− allele was associated with nearly a 50% lower mean serum DBH enzyme activity (nondeletion homozygotes: 41.02 ± 24.34 IU/liter; heterozygotes: 32.07 ± 18.10 IU/liter; and deletion homozygotes: 22.31 ± 13.48 IU/liter in a pooled sample of mothers and controls. These researchers suggest that lowered maternal serum DBH activity results in a suboptimal uterine environment (decreased norepinephrine relative to dopamine), which, in conjunction with genotypic susceptibility of the fetus, results in autism spectrum disorder in some families.

Unfortunately, the children with autism of these mothers with low DBH were not tested for low DBH. However, a high incidence of hyperflexible joints and postural hypotension has been reported in autism, implicating a potential role of DBH deficiency as a cause of autism.

Dopamine: What is it and how does it impact our body?


Dysautonomia is an encompassing term used to describe several different medical conditions that cause a malfunction of the autonomic nervous system, the system that controls the automatic functions of the body that we do not consciously think about, such as heart rate, blood pressure, digestion, dilation and constriction of the pupils of the eye, kidney function, and temperature control.

The autonomic nervous system consists of two main divisions: the sympathetic nervous system and the parasympathetic nervous system.

The sympathetic nervous system is often considered the “fight or flight” system, while the parasympathetic nervous system is often considered the “rest and digest” or “feed and breed” system.

In many cases, both of these systems have “opposite” actions where one system activates a physiological response and the other inhibits it.

At the effector organs, sympathetic ganglionic neurons release noradrenaline (norepinephrine) while parasympathetic postganglionic neurons release acetylcholine as the main neurotransmitter. If DBH is deficient in the sympathetic nervous system, the substrate of the enzyme dopamine accumulates while the products of the enzyme, norepinephrine and epinephrine, decrease.


Thomas and his colleagues (1995, 1997) found that in pregnant female mice that lacked DBH virtually all progeny died and that there were many deaths of progeny even in pregnant mice with only a single copy of the DBH gene.

By providing additional norepinephrine from the Droxidopa drug to the pregnant mice with the genetic deficiency, the excess mortality of the offspring was prevented. Although not yet reported in humans, DBH deficiency may also be a cause of infertility in humans.



A patient, Megan, first experienced troubling symptoms as a child. Whenever she stood up for more than two minutes, she collapsed. She had similar problems whenever she stood up in church or school.

She suffered from bed wetting when she was older and also had droopy eye lids, excessive bladder infections, and hypoglycemic (low blood sugar) episodes that left her shaky.

As she became a teenager she suffered from severe exercise intolerance and was unable to walk a block or climb a flight of stairs without resting.

She consulted many physicians with no answers. Her standing blood pressure was extremely low (50/30). All of Megan’s symptoms were due to a deficiency of DBH.

After finally finding a physician who diagnosed her with dopamine beta hydroxylase deficiency, she was put on the drug Northera® which was able to form norepinephrine by circumventing the impaired DBH pathway as illustrated (on the right).

In 2010, after taking Northera®, Megan completed an Olympic-length triathlon: a 1,500-meter swim, 40-kilometer bike ride, and 10K run. “For me it was like checking this off the list,” Megan says. “I was so grateful for my new physicality.”

Droxidopa (Northera®) has been used as a replacement for norepinephrine and has been effective in treating other disorders besides POTS and hypotension, such as autism and Parkinson’s disease.


Dysautonomia is not rare. Over 70 million people worldwide live with various forms of dysautonomia. People of any age, gender or race can be impacted. POTS is estimated to impact a total of 1,000,000 to 3,000,000 Americans.

The condition can cause light headness, fainting, tachycardia, chest pains, shortness of breath, GI upset, shaking, exercise intolerance, temperature sensitivity and more.

While POTS predominantly impacts young women who look healthy on the outside, researchers compare the disability seen in POTS to the disability seen in conditions like COPD and congestive heart failure.


The following substances may interfere in the test and cause lower results:

  • Clostridia metabolites
  • Phenols
  • Fusaric acid from Fusarium mold species
  • Antabuse®
  • Nutrasweet® (aspartame)