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

Conditions necessary for outbreak of staphylococcal food, Q. Conditions Nec...

Q. Conditions Necessary for Outbreak of staphylococcal food? The following conditions are necessary for an outbreak of staphylococcal food poisoning: 1. The food must conta

Diaphragmatic hernia, Diaphragmatic Hernia: In this condition there  i...

Diaphragmatic Hernia: In this condition there  is a slight herniation ofabdominal organs  (stomach intestine and liver) or extreme protrusion of abdominal contents into the th

Define methods used in combination with pasteurization, Define methods used...

Define methods used in combination with pasteurization? Typical other methods used in combination with pasteurization include: 1.  Refrigeration as in the case of   milk

Explain prevalence in coronaw artery disease, Explain Prevalence in coronaw...

Explain Prevalence in coronaw artery disease ? The first report to highlight the high prevalence of CAD among Indian expatriates came from an autopsy study done in Singapore. C

Neurites, NEURITE S - The processes of neurons are called neurites. Th...

NEURITE S - The processes of neurons are called neurites. These are of two types: dendrites or dendrons and an axon or axis cylinder or neuraxon. (a)      Dendrites (Den

Immunolocalization, uses and advantages and disadvantages of immunolocaliza...

uses and advantages and disadvantages of immunolocalization

What do you mean by st hystersis, Q. What do you mean by ST Hystersis? ...

Q. What do you mean by ST Hystersis? Ans. The rate of recovery of ST depression has been recognized as a measure of ischaemia severity for many years. When comparing the m

Define proteins required for underweight - nutritional care, Define Protein...

Define Proteins required for underweight - Nutritional Care? Proteins are required for tissue building, as well as, to take care of the daily wear and tear. Under weight indivi

Kingdom protista, what is the taxonomy of phylum sporoza?

what is the taxonomy of phylum sporoza?

Pisces, weberian oscles found in

weberian oscles found in

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