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Biochemistry

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Lipid

Question
3 out of 63
 

LEIGH disease is due to the accumulation of



A Glycogen
B Pyridoxine

C Sphingomyelin
D Lipofuscin

Ans. A glycogen

1. Leigh’s disease or Subacute Necrotizing Encephalopathy

a. Is characterized by an arrest of psychomotor development, feeding problem, seizures, extraocular

b. palsies, and weakness with hypotonia between one and 2 years of age.

c. The cardinal Laboratory finding is lactic Acidemia.

d. Various biochemical abnormalities have been found, all of which lie in the mitochondrial

e. pathway for converting pyruvate to ATP.

f. It is an eg. of Mitochondrial Encephalopathiesthat leads to abnormalities of basal ganglia

g. pyramidal sign and Brain stem.

h. Four known genetically determined cause of LEIGH disease -

i. Pyruvate dehydrogenase complex deficiency X-linked transmission

ii. Complex IV COX deficiency AR transmission

iii. Complex I deficiency and

iv. Complex V ATPase deficiency maternal transmission

Now it is clear from above that something abnormities of metabolism of glucose so, the glycogen

sinceLactate, and Pyruvate Level

Sphingo myelin deposited in Niemann-Pick disease, due to deficiency of sphingomyelins.

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