Bioch...Q7 - maryam2009

[ArchivalUser]

A child with a congenital deficiency of medium-chain acyl CoA dehydrogenase activity .Which of the following signs or symptoms would most likely occur upon fasting in this child?

A.Hypolacticacidemia
B.Ketoacidosis
C.Hyperglycemia
D.Dicarboxylic acidosis
E.Hyperchylomicronemia

[ArchivalUser]

e...

[ArchivalUser]

I know it would cause hypoketotic hypoglycemia.
Answer: D?

[ArchivalUser]

increased levels of dicarboxylic acid, low glucose and low ketons

[ArchivalUser]

by what mechanism .... plz explain @lovetest or sairakhan

[ArchivalUser]

due to fatty acid degradation defect. You can't form the end products which are ketone bodies and TCA cycle

[ArchivalUser]

ya...but the accumulations would be the medium chain fatty acyl coA molecules right ? why dicarboxylic acids ?

[ArchivalUser]

dd

[ArchivalUser]

D.. is the correct answer

another name for MCAD deficiency is Non-ketotic hypoglyemia.The halmarks of this deficiency(hepatic beta oxidation deficiency)include:

profound fasting hypoglcemia
low to absent ketons
lethargy,coma...death if untreated
C8-C10 acyl carnitines in blood
**DS : Dicarboxylic acidosis in blood and urine
cH3-(cH2)n-cooH.............>cooH-(cH2)n-cooH

[ArchivalUser]

@berserker

due to the MCAD deficiency ..medium chain fatty acids will be accumulated in peroxisomes and will be oxidized by ω-oxidation system to dicarboxylic acids that serve as substrates for peroxisomal β-oxidation.
......................... ω-oxidation .....................
cH3-(cH2)n-cooH.............>cooH-(cH2)n-cooH