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An arylsulfatase is any of a group of sulfatase enzymes.
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What kind of inherited condition is Metachromatic leukodystrophy?
Metachromatic leukodystrophy (MLD), also called sulfatide lipidosis and arylsulfatase A (ARSA) deficiency, is inherited as an autosomal recessive trait, due to mutations in the arylsulfatase A (ARSA) gene
What role does the ARSE gene play in the human body?
The ARSE gene (also known as: "Arylsulfatase E", with its symbols "ASE", "CDPX", "CDPX1" or "CDPXR") is linked to the development of both bones and cartilage in the human body.
Metachromatic leukodystrophy?
DefinitionMetachromatic leukodystrophy (MLD) is a genetic disorder that affects nerves, muscles, other organs, and behavior. It slowly gets worse over time.Alternative NamesMLD; Arylsulfatase A deficiency; Leukodystrophy - metachromaticCauses, incidence, and risk factorsMLD is usually caused by the lack of an important enzyme called arylsulfatase A. Because this enzyme is missing, chemicals called sulfatides build up in and damage the nervous system, kidneys, gallbladder, and other organs. In particular, the chemicals damage the protective sheaths that surround nerve cells.The disease is passed down through families (inherited). You must get a copy of the defective gene from both your parents to have the disease. Parents can each have the defective gene, but not have MLD. A person with one defective gene is called a "carrier."Children who inherit only one defective gene from one parent will be a carrier, but usually will not develop MLD. When two carriers have a child, there is a 25% chance that the child will get both genes and have MLD.MLD occurs in about 1 in 40,000 people. There are three forms of the disease. They are based on when the symptoms begin:Late infantile MLD symptoms usually begin by ages 1 - 2.Juvenile MLD symptoms usually begin between ages 4 and 12.Adult (and late-stage juvenile MLD) symptoms may occur between age 14 and adulthood (over age 16), but may begin as late as the 40s or 50s.SymptomsAbnormal high muscle tone, abnormal muscle movementsBehavior problemsDecreased mental functionDecreased muscle toneDifficulty walkingFeeding difficultiesFrequent fallsInability to perform normal tasksIncontinenceIrritabilityLoss of muscle controlNerve function problemsPersonality changesPoor school performanceSeizuresSpeech difficulties, slurringSwallowing difficultySigns and testsSigns include:Abnormal eye movementsAbnormal postureDecreased or no deep tendon reflexesComaOptic nerve atrophyPossible tests include:Blood or skin culture to look for low arylsulfatase A activityBlood test to look for low arylsulfatase A enzyme levelsCT scanDNA testing for the ARSA geneMRINerve biopsyNerve velocity conduction studiesUrinalysisUrine chemistryTreatmentThere is no cure for MLD. Care focuses on treating the symptoms and preserving the patient's quality of life with physical and occupational therapy.Research is studying techniques to replace the missing enzyme (arylsulfatase A).Support GroupsFor additional information and resources, see:United Leukodystrophy Association - www.ulf.orgMLD Foundation - www.mldfoundation.orgExpectations (prognosis)MLD is a severe disease that gets worse over time. Eventually people lose all muscle and mental function. Life span varies depending on what age the condition started, but the disease course usually runs 3 - 20 or more years.People with this disorder are expected to have a shorter-than-normal lifespan. The earlier the age at diagnosis, the more quickly the disease progresses.PreventionGenetic counseling is recommended if you have a family history of this disorder.
Is lactose milk a dairy product?
In the food and beverage industry of Western Europe, a significant customer change towards organic food and beverages has been observed in recent years. This change is mainly due to raising consumer perception of unethical and synthetic ingredients in non-organic food and beverage products. This is the precise reason why the production of organic milk in this area is strictly regulated and even the selling of milk requires a mandatory licence from the relevant authorities. Consumers have faith, because of these laws, that everything they consume has been well inspected. This gives producers the requisite incentive to go beyond other non-organic products into organic lactose-free dairy products. The worldwide demand for lactose-free dairy products is mainly driven by the growing prevalence of lactose intolerance across the world. Furthermore, the rising demand for healthier alternatives to milk would cause the market to expand significantly in the coming years.Speaking of key problems faced, taste has remained a persistent source of customer concern. Because of arylsulfatase present in them, lactose free dairy products may have an unpleasant taste. This might to a degree hamper the growth prospects of the market. Source: Future Market Insights Reports-Lactose Free Dairy Products Market
