AGAS - Overview: Alpha-Galactosidase, Serum (2023)

FAQs

What is a normal alpha-galactosidase level? ›

Interpretation. In male patients, results less than 1.2 nmol/mL/hour in properly submitted specimens are consistent with Fabry disease. Normal results (≥1.2 nmol/mL/hour) are not consistent with Fabry disease.

This is a screening test for Fabry Disease. If patient is NOT KNOWN to have Fabry Disease, use the DBS kit. Patients will not be charged for this test if the DBS (Sanofi Genzyme) kit is used.

What is Fabry disease? Fabry disease (also known as alpha-galactosidase-A deficiency) is an inherited neurological disorder that occurs when the enzyme alpha-galactosidase-A cannot efficiently break down fatty materials known as lipids into smaller components that provide energy to the body.

Alpha-galactosidase reduced gas production following a meal rich in fermentable carbohydrates and may be helpful in patients with gas-related symptoms.

beta-galactosidosis is a lysosomal storage disorder caused by a deficiency of acid beta-galactosidase, including to autosomal recessive diseases; GM1-gangliosidosis (neurovisceral form) and Morquio B disease (skeletal form).

Persons with at least 1 alpha-gal IgE test result greater than or equal to 0.1 kU/L were considered positive. Persons with all alpha-gal IgE test results less than 0.1 kU/L were considered negative.

For instance, magnesium stearate and gelatin are found in formulations of acetaminophen, naproxen, lisinopril, clonidine, and hydrocodone, and allergic reactions to these medications have been potentially linked to alpha-gal.

A blood test can confirm and measure the amount of alpha-gal antibodies in your bloodstream. This is the key test for diagnosis of alpha-gal syndrome. A skin test. A health care provider pricks your skin and exposes it to small amounts of substances taken from commercial or fresh red meat.

Serious side effects

Signs of an allergic reaction, like rash; hives; itching; red, swollen, blistered, or peeling skin with or without fever; wheezing; tightness in the chest or throat; trouble breathing, swallowing, or talking; unusual hoarseness; or swelling of the mouth, face, lips, tongue, or throat.

What foods does alpha-galactosidase help with? ›

Alpha-galactosidase can help break down a type of non-absorbable fiber called galactooligosaccharides (GOS), mostly found in beans, root vegetables and some dairy products. GOS is known to be beneficial, but it can cause gas and bloating in some individuals.

A total of 7 adverse events were reported: mild constipation and diarrhea (reported by 2 patients during both treatments), moderate abdominal pain (reported by 1 patient during alpha-galactosidase treatment), and moderate constipation (reported by 2 patients taking active charcoal).

The α-galactosidase A gene (GLA) is located on the long arm of the X chromosome, in position Xq22, spans approximately 12 kb of genomic DNA, and contains seven exons (Kornreich et al., 1989). The complementary DNA is 1290 bases and encodes a polypeptide of 429 amino acids including a 31 amino acid signal peptide.

In summary, alpha galactosidase assists in reducing digestive issues, such as gas and bloating, by targeting food particles that are hard to digest (i.e. glycolipids and glycoproteins) before they can accumulate and become fermented by beneficial microorganisms in the intestines.

β-Galactosidase is found in bacteria, fungi, and yeasts. In plants, it is mainly found in almonds, peaches, apples, and apricots.

Human β-d-galactosidase (EC 3.2. 1.23, β-Gal) is a lysosomal enzyme that catalyzes the hydrolysis of terminal β-galactose residues from various substrates, including ganglioside G_M1³ and keratan sulfate (1–3).

β-Galactosidase is an enzyme produced by some strains of lactic acid bacteria (LAB) commonly found in dairy products; however, industrial demand for these enzymes is still low. Acid whey (AW), a lactose-rich byproduct, has large output from cottage cheese and remains unexploited.

Apple cider vinegar is loaded with enzymes that aid in digestion. While you can take digestive enzymes as a supplement, drinking apple cider vinegar may offer similar benefits for less money.

Alpha-galactosidase converts some sucrose into glucose. This can affect your blood sugar level.

What are the 4 main digestive enzymes? ›

The 4 main digestive enzymes are carbohydrases, proteases, lipases, and nucleases.

Furthermore, some patients reported a wide range of mental health issues, such as anxiety, depression, fatigue, poor memory, irritability, sleep disturbances, etc. Additionally, alpha-Gal allergy symptoms may closely resemble those of IBS.

The Experts. If you can, make an appointment with an allergist who is an expert on alpha-gal syndrome.

At the time this is being written, antibody levels > 0.10 kU/L are typically considered a positive test result (10). Antibody levels >2 IU/ml or >2% of total IgE make the diagnosis of alpha-gal syndrome very likely (1). As with any allergy, false negatives also occur, although they are less common.

Alpha-gal syndrome (AGS) (also called alpha-gal allergy, red meat allergy, or tick bite meat allergy) is a serious, potentially life-threatening allergic reaction. AGS symptoms occur after people eat red meat or are exposed to other products containing alpha-gal. AGS can have life-changing effects.

Treating and preventing alpha-gal allergy

Allergic reactions to alpha-gal can be treated with an over-the-counter antihistamine such as diphenhydramine (Benadryl).

Alpha-gal syndrome can cause a serious allergic reaction called anaphylaxis. It can be deadly without treatment. Anaphylaxis is treated with prescription medicine called epinephrine, also known as adrenaline. You can give yourself a shot of epinephrine with a device called an auto-injector (EpiPen, Auvi-Q, others).

The theory is that blood type B may share certain structural similarities with alpha gal causing those with B type blood to ignore the transferred substance since it is similar to their own tissue.

Treatment for alpha-gal (AGS) syndrome typically involves avoiding foods with alpha-gal and taking medications to manage symptoms that may occur with accidental exposure. Your healthcare provider can work with you to help you avoid products and medications containing alpha-gal.

Is alpha-galactosidase the same as alpha gal? ›

The enzyme α-galactosidase (α-GAL, also known as α-GAL A; E.C. 3.2. 1.22) is responsible for the breakdown of α-galactosides in the lysosome. Defects in human α-GAL lead to the development of Fabry disease, a lysosomal storage disorder characterized by the buildup of α-galactosylated substrates in the tissues.

Dosage & When it is to be taken

The recommended dosage for alpha-galactosidase is 260 - 3000 FCC (Food Chemical Codex) units per day.

In our study, patients with alpha-gal meat allergy developed GI symptoms—most commonly episodic abdominal pain, nausea, and diarrhea—without more traditional manifestations of food allergy such as hives, angioedema, or anaphylaxis.

Symptoms of enzyme deficiency tend to first show up in the gut. That's why you typically see digestive issues with insufficient enzyme levels like bloating, gas, diarrhea, constipation, and undigested food in stools. If your body doesn't have enough digestive enzymes, it's unable to break down foods properly.

Taking too many digestive enzymes can lead to symptoms of digestive discomfort, including: Constipation. Diarrhea. Abdominal cramps.

Of course, you can choose to avoid foods that give you trouble. But taking alpha-galactosidase or lactase supplements to help with digestive issues is safe and evidence-based.

Lipase is a digestive enzyme that boosts the absorption of fat in your body by breaking it down into glycerol and free fatty acids ( 9 ). Some studies show that supplementing with lipase may decrease feelings of fullness ( 10 , 11 ).

Upset stomach, stomach pains and cramping. Diarrhea. Nausea. Gas and bloating.

Enzymes are essential for digestion, liver function and much more. Too much or too little of a certain enzyme can cause health problems. Enzymes in our blood can also help healthcare providers check for injuries and diseases.

Two recombinant forms of human α-galactosidase are called agalsidase α (INN) and agalsidase β (INN).

What sugars does alpha-galactosidase break down? ›

The hydrolytic enzyme alpha-galactosidase (α-GAL) can break down RFOs into sucrose and galactose releasing the monosaccharide α-galactose back into the system.

Usual Adult Dose for Flatulence

Take 5 drops/problem food right before your first bite. A typical meal has 3 servings of problem food. Alpha-D-galactosidase oral tablet/capsule, chewable: Chew or swallow whole 1 tablet/capsule per problem food right before your first bite.

How many Beano can you take per day? You can take Beano with every meal. You can take a double dose if you are eating larger than typical serving sizes of problematic foods. As a digestive enzyme, there is no upper limit for the amount of Beano you can safely take in a day.

Of course, you can choose to avoid foods that give you trouble. But taking alpha-galactosidase or lactase supplements to help with digestive issues is safe and evidence-based.

Definition of Activity Alpha Galactosidase

One unit of alpha galactosidase activity (U) is defined as the amount of enzyme which liberates 1 millimole of paranitrophenol per minute from 5 mmol/L of paranitrophenol-α-D-galactopyranose solution at pH 5.5 and 37 ℃.