Heme Flashcards

1
Q

Biosynthesis of heme takes place in

A

Almost all tissues
Synthesized in normoblast
But not in mature erythrocytes

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2
Q

Where does the biosynthesis of purine take place

A

Partly both
Cytoplasm
Mitochondria

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3
Q

Types or forms of alas

A

Erythroid and hepatic/non erythroid

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4
Q

Basic administration of what can help porphyria

A

Glucose

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5
Q

Where is ferrochelatase located

A

Mitochondria

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6
Q

Pyrrole rings joined together by what bridges

A

Methylene bridges

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7
Q

The methylene bridges that connect pyrrole rings are derived from

A

Alpha carbon of glycine

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8
Q

Deficiency of ALAS 2 results in what disorder and why

A

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.

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9
Q

What are sideroblasts

A

Sideroblasts are erythroblasts with non-heme iron-containing organelles, called siderosomes.

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10
Q

What is porphyria

A

Characterized by increased production and excretion of porphyrins or their precursors (ALA or PBG )

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11
Q

Grouping of porphyrias

A

Hepatic
Erythropoietin
With both abnormalities

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