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tokes in - mitochondria .

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enzyme

-
PBG -

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3. step 1 : 4 ✗ PBG ˢ4n the se

g hydroxy methyl bi /on cyclic

µ
.

PBG de Ami nose .

↳ step 2 : hydroxy methyl bildne É uroporphyinogen II

↳
synthesise

step } : uroporphynogen# g coprophyrinogen#

4. → step 1 : coproporphyinogenllt És protoporphyrin IX

↳ step 2 :
proto porphyrin It + iron ferrochelotoseg home
 • ferrochelotose :

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diagnostic value .

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-
micro cytic RBCs .

-
hypochromia RBCs .
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↳ because of the interaction between zinc + pp Ring .

• severe iron deficiency + genetic defects in .

ferrocheldtose

have these three chordchtdristics as well . .

④
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• mis - sense mutation in ⊕ a promoter region mutation

obiliruben level Rises to a huge amount & bilirubin level = 2 -3mg/do

= 30 -60 mg / dc → !! 1 Mfi is

condition benign condition

•
very dangerous . • .

✗ potient usually dies within 1Yeor . • patient lives pedcetully .

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