Explain about maple syrup urine disease, Biology

Assignment Help:

Q. Explain about Maple Syrup Urine Disease?

Maple Syrup Urine Disease (MSUD) is a group of inherited metabolic disorders of three branched chain amino acids (BCAA) namely leucine, isoleucine and valine. These three amino acids are normally metabolised to ketoacids and then decarboxylated lo simple acids. In MSUD disorder an oxidative decarboxylase in the white blood cells is missing. Since the carboxyl groups cannot be removed there is an accumulation of branched chain ketoacids and their amino acid precursors. The branched chain a ketoacids is relatively acute neurotoxins and probably interfere with oxygen consumption and ATP production in the medullar reticular substance of the brain. There is a progressive neurologic dysfunction and production of fragrant urine with the odour of burnt sugar or maple syrup.


Related Discussions:- Explain about maple syrup urine disease

Anticoagulant therapy, Anticoagulant therapy To prevent blood clottin...

Anticoagulant therapy To prevent blood clotting on the valve, patients with prosthetic valves require life long anticoagulant therapy. The patient is put on oral antico

Explain advantages and disadvantages of spread plate method, Explain Advant...

Explain Advantages and Disadvantages of Spread Plate Method? The advantages and disadvantages of spread plate method are highlighted next. Advantages   1. It is useful fo

Illustrate about the neuropsychological assessment, Illustrate about the ne...

Illustrate about the neuropsychological assessment A neuropsychological assessment is a clinical examination of both the working brain and dysfunctional brain. Neuropsychologic

Subatomic Particles, We have end of semester exams coming up meaning that a...

We have end of semester exams coming up meaning that a majority of it will be over subatomic particles

Show signs and symptoms and treatments of hepatitis a, Q. What are the mode...

Q. What are the modes of transmission, main signs and symptoms and treatments of hepatitis A? The Hepatitis A is an acute disease of low mortality caused by the hepatitis A vir

Oil seed meals as source of limiting amino acids, Oil seed meals as source ...

Oil seed meals as source of limiting amino acids Generally ruminants are not perceived to have a requirement for essential amino acids as the rumen microbes can synthesis esse

What are the lateral lines of fishes, What are the lateral lines of fishes?...

What are the lateral lines of fishes? The lateral lines of bony fishes are sense organs that extend along both sides of the animal body. They make contact with the environment

Hydra, hydra has what symmetry......radial or biradial????

hydra has what symmetry......radial or biradial????

Explain huntington disease gene, Research methods used in studying a geneti...

Research methods used in studying a genetic disease change with technological advances. These changes can be seen in Reading 22.1: Discovering the Huntington Disease Gene, which de

Define the food processing, Define the Food Processing? We are all awar...

Define the Food Processing? We are all aware that delay in the use of fresh foods, alters its freshness, palatability and nutritive value. Therefore, it becomes very important

Write Your Message!

Captcha
Free Assignment Quote

Assured A++ Grade

Get guaranteed satisfaction & time on delivery in every assignment order you paid with us! We ensure premium quality solution document along with free turntin report!

All rights reserved! Copyrights ©2019-2020 ExpertsMind IT Educational Pvt Ltd