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

Define nitrogen estimation - kjeldahl''s method, Define Nitrogen Estimation...

Define Nitrogen Estimation - Kjeldahl's Method? Nitrogen in the protein is estimated by a method referred to as Kjeldahl's method. Although over a period of time many other met

What are the respective constituents of the gray matter, What are the respe...

What are the respective constituents of the gray matter and of the white matter of the spinal cord? The gray matter, or gray substance, of the spinal cord having predominantly

Human milk composition and infant growth and development, Explain the Human...

Explain the Human Milk Composition and Infant Growth and Development? This sub-section deals with composition of human milk, compares human milk with cow's milk and why human m

Determine the width of attached gingiva at implant site, Width of Attached ...

Width of Attached Gingiva at Implant Site The zone of attached gingiva needs to be intact and of adequate width to protect (by separating) the tissue around the implant. If it

Congestive heart failure (chf), C o ngestive heart failure (CHF) The ...

C o ngestive heart failure (CHF) The heart is unable to maintain circulatory equilibrium at rest and congestion of venous circuit takes place followed by dilatation of vessel

Plantae, significance of amoebozoa

significance of amoebozoa

Reproduction, The fusion of two individuals with each acting as a gamete is...

The fusion of two individuals with each acting as a gamete is known as

Difference between the concepts of karyotype and genome, What is the differ...

What is the difference between the concepts of karyotype and genome? Genome is the set of DNA molecules that characterizes each living being or every species. The concept then

Musculoskeletal system, MUSCULOSKELETAL SYSTEM: Disease of  the skin  ...

MUSCULOSKELETAL SYSTEM: Disease of  the skin  and musculoskeletal  system occur commonly  in  early age group. There are  not many  statistics  to prove the exact frequency of

Describe the technique of food processing, Technique of food processing ...

Technique of food processing Freezing is another ancient technique of food processing, which allows the transportation of perishable food items for long distances from producti

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