Heme Flashcards
Biosynthesis of heme takes place in
Almost all tissues
Synthesized in normoblast
But not in mature erythrocytes
Where does the biosynthesis of purine take place
Partly both
Cytoplasm
Mitochondria
Types or forms of alas
Erythroid and hepatic/non erythroid
Basic administration of what can help porphyria
Glucose
Where is ferrochelatase located
Mitochondria
Pyrrole rings joined together by what bridges
Methylene bridges
The methylene bridges that connect pyrrole rings are derived from
Alpha carbon of glycine
Deficiency of ALAS 2 results in what disorder and why
XLSA
ALAS2 gene on X chromosome. X-linked sideroblastic anemia, XLSA.
Sideroblasts are erythroblasts with non-heme iron-containing organelles, called siderosomes. XLSA has also been called congenital sideroblastic anemia, hereditary sideroblastic anemia, hereditary iron-loading anemia, X-linked hypochromic anemia, hereditary hypochromic anemia, and hereditary anemia.
What are sideroblasts
Sideroblasts are erythroblasts with non-heme iron-containing organelles, called siderosomes.
What is porphyria
Characterized by increased production and excretion of porphyrins or their precursors (ALA or PBG )
Grouping of porphyrias
Hepatic
Erythropoietin
With both abnormalities