Page 292 - Biochemistry PharmD General

2. α-thalassemia:
α-thalassemia occurs due to the decreased rate of
synthesis of α-chain of globin. But this is rarely seen

due to the presence of two pairs of genes for a chain

in the Hb gene.

Ghicose-6-Phosphate Dehydrogenase Deficiency:

• It is an X-linked recessive hereditary disease

characterised by abnormally low levels of the

glucose-6-phosphate dehydrogenase enzyme

(abbreviated G6PD or G6PDH).

• Glucose-6-phosphate dehydrogenase (G6PD) is an

enzyme in the pentose phosphate pathway, a

metabolic pathway that supplies reducing energy to

cells by maintaining the level of the co-enzyme

nicotinamide adenine dinucleotide phosphate

(NADPH). 291