Complex l or NADH dehydrogenase in the inner mitochondrial membrane transfers el

Question

Complex l or NADH dehydrogenase in the inner mitochondrial membrane transfers electrons from NADH to ubiquinone (Coenzyme Q). What other important function is carried out by this complex? Complex l can also transfer electrons to the mitochondrial inner membrane space Complex l pumps four protons (H+) from the mitochondrial matrix into the inner membrane space for every pair of electrons transferred Complex l pumps four protons (H+) from the mitochondrial inner membrane space into the matrix for every pair of electrons transferred The electrons transferred by complex l are used directly to reduce oxygen to water Cori's disease, a type llla glycogen storage disease, results in an enlarged liver in infants and muscle damage. These symptoms are a result of a mutation in an important enzyme in glycogen metabolism. When the liver cells are analyzed the glycogen present is only partially broken down and there are a large number of nonreducing ends remaining in the glycogen particle. What enzyme is defective in this disease? Glycogen synthase Phoshoglucomutase Debranching enzyme Branching enzyme

Explanation / Answer

Que 7:

NADH dehydrogenase (ubiquinone) is the first enzyme of ETC belongs to enzyme of Oxidoreductase family, responsible for the coupling of transfer of proton in electrogenic way. It is one of the largest and most complicated enzymes of ETC. The whole reaction can be written as follows:

NADH + H+ + CoenzymeQ + 4H+in NAD+ + CoQH2 + 4H+out

Therefore, the complex I is responsible for the transfer 1 electron pair against the transfer of 4 H+ and thus option 3 is correct.

Que 8

Cori’s disease is result of mutation in a gene AGL, responsible for the synthesis of an enzyme, known as ‘glycogen debranching enzyme’. This enzyme is responsible for the breakdown of glycogen. Since, the glycogen is major type of animal reserve food material, therefore, hampering its breakdown cause serious damage to liver. Thus, option C is correct.

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