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How is maple syrup urine syndrome inherited?

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It is an autosomal recessive condition.

MSUD is an autosomal-recessive disorder, with an incidence of 1 in 120 000 to 1 in 500 000. It is highly prevalent in the inbred Mennonite population in Pennsylvania, occurring in approximately 1 in 176 newborns. In countries where consanguineous marriages are common the frequency is also higher (about 1 in 50 000 in Turkey). About 75% of those affected suffer from the severe classic form, and the remainder suffer from the milder intermediate or intermittent variants. Over 150 different causal mutations scattered among the three E1α, E1β, and E2 genes give rise to either classic or intermediate clinical phenotypes.
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What is maple syrup?

Maple syrup is a sugary, sticky, molasses-type condiment made from Maple Tree sap. The sap is collected in pails by tapping trees (drilling holes in the trunk and letting the

When was Maple Syrup Urine Disorder discovered?

Maple Syrup Urine Disease (MSUD) was discovered by John Menkes in 1954. This is a disorder whereby there is a disruption in the metabolism of branched-chained amino acids.

How does Maple syrup urine Disease occurs?

MSUD occurs when a persons metabolism cannot break down certain proteins and amino acids. These chemicals, especially, are leucine, isoleucine, and valine. In a result of this

Is Maple syrup urine disease dominant?

MSUD is an autosomal-recessive disorder, with an incidence of 1 in 120 000 to 1 in 500 000. It is highly prevalent in the inbred Mennonite population in Pennsylvania, occurrin

How can you get maple syrup from a maple tree?

A hole is drilled into the tree in early spring and is fitted with tubing and/or buckets to collect the sap as it rises in the tree. The sap is boiled down into syrup. Forty g

How harmfal is maple syrup urine?

Well over 100 inborn errors of metabolism can now be detected by newborn screening, with more certainty. Concomitantly with the onset of the symptoms, the patient affected wit

How is Maple Syrup Urine Disease inherited?

MSUD is an autosomal-recessive disorder, with an incidence of 1 in 120 000 to 1 in 500 000. It is highly prevalent in the inbred Mennonite population in Pennsylvania, occurrin